Page Comparison

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, [ipNF04.4], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 77bdc425-5f93-4fc0-9033-a3c63aa6da73, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 6c7d59b3-e542-40f3-9d5f-04ca85466b26, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, a8f48ce7-438c-4564-a70b-020abc96d5fe, hTERT NF1 ipNF00.6, cellLine, RRID:CVCL_UI76, Female, Homo sapiens, null, null, [ipNF00.6], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, a8e596b4-6067-4ac2-8d9c-7309f4b391ad, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c3fb3ba5-903b-4b31-a4f1-03462e18093f, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, f21cb7db-ee85-48a7-8f41-e4603238bede, hTERT NF1 ipNF03.3, cellLine, RRID:CVCL_UI77, Male, Homo sapiens, null, null, [ipNF03.3], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, [ipNF06.2A], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[The neurofibroma, a common feature of neurofibromatosis type 1 (NF1), is a benign peripheral nerve sheath tumor that contains predominantly Schwann cells (SC). There are reports that neurofibroma growth may be affected by hormonal changes, particularly in puberty and pregnancy, suggesting an influence by steroid hormones. This study examined the effects of estrogen and progesterone on proliferation and apoptosis in a panel of NF1 tumor xenografts. SC-enriched cultures derived from three human NF1 tumor types (dermal neurofibroma, plexiform neurofibroma, and malignant peripheral nerve sheath tumor (MPNST)) were xenografted in sciatic nerves of ovariectomized scid /Nf1-/+ mice. At the same time, mice were implanted with time-release pellets for systemic delivery of progesterone, estrogen or placebo. Proliferation and apoptosis by the xenografted SC were examined two months after implantation, by Ki67 immunolabeling and TUNEL. Estrogen was found to increase the growth of all three MPNST xenografts. Progesterone was associated with increased growth in two of the three MPNSTs, yet decreased growth of the other. Of the four dermal neurofibroma xenografts tested, estrogen caused a statistically significant growth increase in one, and progesterone did in another. Of the four plexiform neurofibroma SC xenografts, estrogen and progesterone significantly decreased growth in one of the xenografts, but not the other three. No relationship of patient age or gender to steroid response was observed. These findings indicate that human NF1 Schwann cells derived from some tumors show increased proliferation or decreased apoptosis in response to particular steroid hormones in a mouse xenograft model. This suggests that anti-estrogen or anti-progesterone therapies may be worth considering for specific NF1 neurofibromas and MPNSTs., null, null, [Hua Li, Xuelian Zhang, Lauren Fishbein, Frederick Kweh, Martha Campbell-Thompson, George Q Perrin, David Muir, Margaret Wallace], CRL-2885, null, Cancer cell line, 39b4b78a-44f4-4c19-8b62-89ca471fb5d1, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.4161/cbt.10.8.12878, 7cd65ff8-953a-40ed-b318-b8562c646b11, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Cancer Biology & Therapy, [malignant peripheral nerve sheath tumor], yes, null, null, null, null, c21f4170-a611-4062-a455-26e74560a4d4, null, null, b72d99fd-ee1e-42ec-92b6-9f89e375cce1, sNF02.2, cellLine, RRID:CVCL_K280, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, b603b0a5-cc1d-48b3-aa03-e70a7d19eb1e, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, [ipNF05.5 mixed clone, ipNF05.5 (mixed clone)], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, [ipNF05.5], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1 (NF1) and have a risk of malignant progression. Past efforts to establish xenograft models for neurofibroma involved the implantation of tumor fragments or heterogeneous primary cultures, which rarely achieved significant tumor growth. We report a practical and reproducible animal model of plexiform-like neurofibroma by xenograft of an immortal human NF1 tumor-derived Schwann cell line into the peripheral nerve of scid mice. The S100 and p75 positive sNF94.3 cell line was shown to possess a normal karyotype and have apparent full-length neurofibromin by Western blot. These cells were shown to have a constitutional NF1 microdeletion and elevated Ras-GTP activity, however, suggesting loss of normal neurofibromin function. Localized intraneural injection of the cell line sNF94.3 produced consistent and slow growing tumors that infiltrated and disrupted the host nerve. The xenograft tumors resembled plexiform neurofibromas with a low rate of proliferation, abundant extracellular matrix (hypocellularity), basal laminae, high vascularity, and mast cell infiltration. The histologic features of the developed tumors were particularly consistent with those of human plexiform neurofibroma as well. Intraneural xenograft of sNF94.3 cells enables the precise initiation of intraneural, plexiform-like tumors and provides a highly reproducible model for the study of plexiform neurofibroma tumorigenesis. This model facilitates testing of potential therapeutic interventions, including angiogenesis inhibitors, in a relevant cellular environment., null, null, [George Q Perrin, Lauren Fishbein, Susanne A Thomson, Stacey L Thomas, Karen Stephens, James Y Garbern, George H DeVries, Anthony T Yachnis, Margaret R Wallace, David Muir], CRL-2886, null, Cancer cell line, 57f88a46-b615-41fa-9b89-9cdbc9dff0dc, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1002/jnr.21226, 362b5ed5-90bd-47b6-89d7-0093f1b79204, null, null, null, https://www.atcc.org/, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Journal of Neuroscience Research, [malignant peripheral nerve sheath tumor], yes, null, null, null, null, 52529f5e-062b-40c9-9e87-ec52fe0ef918, null, null, 504647eb-6b60-492a-bb51-3ab025830f51, sNF94.3, cellLine, RRID:CVCL_K164, Female, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, e7389739-edb8-4e6c-9ad6-06bfb6b3db0e, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, [ipNF95.11b C/T, ipNF95.11bC_T], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, [ipNF95.11b C, ipNF95.11bC], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, [ipNF04.4], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 77bdc425-5f93-4fc0-9033-a3c63aa6da73, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 6c7d59b3-e542-40f3-9d5f-04ca85466b26, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, a8f48ce7-438c-4564-a70b-020abc96d5fe, hTERT NF1 ipNF00.6, cellLine, RRID:CVCL_UI76, Female, Homo sapiens, null, null, [ipNF00.6], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, a8e596b4-6067-4ac2-8d9c-7309f4b391ad, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c3fb3ba5-903b-4b31-a4f1-03462e18093f, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, f21cb7db-ee85-48a7-8f41-e4603238bede, hTERT NF1 ipNF03.3, cellLine, RRID:CVCL_UI77, Male, Homo sapiens, null, null, [ipNF03.3], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, [ipNF06.2A], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, [ipNF05.5 mixed clone, ipNF05.5 (mixed clone)], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, [ipNF05.5], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, [ipNF95.11b C/T, ipNF95.11bC_T], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, [ipNF95.11b C, ipNF95.11bC], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3389, null, Telomerase immortalized cell line, 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94, null, Derived from a plexiform neurofibroma growing on cranial nerve XII., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 108cfda9-d027-49cf-b950-d3e76097aa3c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, b44361f2-9021-4920-901a-b1a1f9143f97, hTERT NF1 ipNF95.6, cellLine, RRID:CVCL_UI70, Male, Homo sapiens, null, null, [ipNF95.6], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, e4426959-f253-4eac-8c68-3415f43104af, ATCC, https://www.atcc.org/]
[Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology. Introduction of a Synapsin I promoter driven Cre transgenic mouse strain into the conditional NF1 background has ablated NF1 function in most differentiated neuronal populations. These mice have abnormal development of the cerebral cortex, which suggests that NF1 has an indispensable role in this aspect of CNS development. Furthermore, although they are tumor free, these mice display extensive astrogliosis in the absence of conspicuous neurodegeneration or microgliosis. These results indicate that NF1-deficient neurons are capable of inducing reactive astrogliosis via a non-cell autonomous mechanism., b75c336e-791d-4a62-b556-153c88b28960, mouse cells, [Y Zhu, M I Romero, P Ghosh, Z Ye, P Charnay, E J Rushing, J D Marth, L F Parada], JAX_017640, https://www.jax.org/strain/017640, null, null, null, [From JAX]: These mice possess loxP sites flanking exons 31-32 of the neurofibromatosis 1 (Nf1) gene and have applications in studies of cancer, neural crest development and neurofibromatosis type I., Neurofibromatosis type 1, 10.1101/gad.862101, be180150-374d-4459-9a81-398c8a34bca9, null, C57BL/6J, UT Southwestern Medical Center, bb909638-4123-41a1-bd61-b300c13e7652, Luis Parada, null, Genes & Development, [plexiform neurofibroma], yes, null, null, null, null, b0332348-464f-4952-a7f7-2ffe4dc6b3f9, null, null, 118aa95e-27f4-48fa-94c7-5f786b8b4f2f, B6.129(Cg)-Nf1tm1Par/J, animalModel, RRID:IMSR_JAX:017640, null, Mus musculus, null, B6.129(Cg)-Nf1tm1Par/J, [Nf1flox], null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, f137e564-d395-4226-bc60-149c3c760e99, The Jackson Laboratory, https://www.jax.org]

[ABSTRACT, ANIMALMODELID, ANIMALSTATE, AUTHORS, CATALOGNUMBER, CATALOGNUMBERURL, CELLLINECATEGORY, CELLLINEID, CONTAMINATEDMISIDENTIFIED, CONTENT_TEXT, DESCRIPTION, DISEASE, DOI, DONORID, GENERATION, GENETICBACKGROUND, ID, INSTITUTION, INVESTIGATORID, INVESTIGATORNAME, INVESTIGATORWEBSITE, JOURNAL, MODELOFMANIFESTATION, MTAREQUIRED, ORCID, ORGAN, ORIGINYEAR, POPULATIONDOUBLINGTIME, PUBLICATIONID, RACE, RESISTANCE, RESOURCEID, RESOURCENAME, RESOURCETYPE, RRID, SEX, SPECIES, STRAINNOMENCLATURE, STRPROFILE, SYNONYMS, TISSUE, TRANSPLANTATIONTYPE, TUMORTYPE, USAGERESTRICTIONS, VENDORID, VENDORITEMID, VENDORNAME, WEBSITE]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, 844b598c-0171-4972-91c3-27aa21b45d52 RRID:CVCL_UI72 hTERT NF1 ipNF05.5 mixed clones ["ipNF05.5 mixed clone", "ipNF05.5 (mixed clone)"] cellLine Derived from a plexiform neurofibroma growing on a hand. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null 9a08aa2d-3d44-497f-9d49-12e9b30d82cf CRL-3387 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 71e33e5d-2392-4112-b284-1386842dd935 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 7, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, ["ipNF05.5 mixed clone", "ipNF05.5 (mixed clone)"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, ab60aae5-7860-4d1d-bb02-208e6631c78b RRID:CVCL_UI68 hTERT NF1 ipNF95.11b C/T ["ipNF95.11b C/T", "ipNF95.11bC_T"] cellLine Derived from a plexiform neurofibroma growing on a brachial plexus. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology c5037bab-6cce-49f6-91ab-2ac26753829b null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 8, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, ["ipNF95.11b C/T", "ipNF95.11bC_T"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, be2333d6-6716-4d13-947d-41f4198497a4 RRID:CVCL_UI67 hTERT NF1 ipNF95.11b C ["ipNF95.11b C", "ipNF95.11bC"] cellLine Derived from a plexiform neurofibroma growing on a brachial plexus. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null f67f594e-b2c0-4497-a5d9-4699b3b2d601 CRL-3390 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 36bd1db8-c1af-4825-a557-13d6276f7949 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 9, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, ["ipNF95.11b C", "ipNF95.11bC"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f RRID:CVCL_UI71 hTERT NF1 ipNF05.5 ["ipNF05.5"] cellLine Derived from a plexiform neurofibroma growing on a hand. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null 071ef5b1-79cb-4a4d-ad61-900eea8f9be0 CRL-3388 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology d43864f1-277e-41dd-ae77-2d8b153b9be8 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 11, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, ["ipNF05.5"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d RRID:CVCL_UI78 hTERT NF1 ipNF04.4 ["ipNF04.4"] cellLine Derived from a plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 c6a7d35b-580c-41ca-bf82-b5bfd5234289 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 4d77247b-d123-42cf-8110-d335d0a9c953 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, 13, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, ["ipNF04.4"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3389, null, Telomerase immortalized cell line, 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94, null, b44361f2-9021-4920-901a-b1a1f9143f97 RRID:CVCL_UI70 hTERT NF1 ipNF95.6 ["ipNF95.6"] cellLine Derived from a plexiform neurofibroma growing on cranial nerve XII. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 108cfda9-d027-49cf-b950-d3e76097aa3c Homo sapiens Male null e4426959-f253-4eac-8c68-3415f43104af CRL-3389 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on cranial nerve XII., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 108cfda9-d027-49cf-b950-d3e76097aa3c, null, null, 14, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, b44361f2-9021-4920-901a-b1a1f9143f97, hTERT NF1 ipNF95.6, cellLine, RRID:CVCL_UI70, Male, Homo sapiens, null, null, ["ipNF95.6"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, e4426959-f253-4eac-8c68-3415f43104af, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb RRID:CVCL_UI74 hTERT NF1 ipNF06.2A ["ipNF06.2A"] cellLine Derived from a pleural plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 10a5e6f2-d331-4b5e-9e43-888cca69d5a9 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology faff6884-47dd-4bbf-ab57-f420d87d456e null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, 15, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, ["ipNF06.2A"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 77bdc425-5f93-4fc0-9033-a3c63aa6da73, null, a8f48ce7-438c-4564-a70b-020abc96d5fe RRID:CVCL_UI76 hTERT NF1 ipNF00.6 ["ipNF00.6"] cellLine Derived from a plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 6c7d59b3-e542-40f3-9d5f-04ca85466b26 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 77bdc425-5f93-4fc0-9033-a3c63aa6da73 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 6c7d59b3-e542-40f3-9d5f-04ca85466b26, null, null, 17, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, a8f48ce7-438c-4564-a70b-020abc96d5fe, hTERT NF1 ipNF00.6, cellLine, RRID:CVCL_UI76, Female, Homo sapiens, null, null, ["ipNF00.6"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, a8e596b4-6067-4ac2-8d9c-7309f4b391ad, null, f21cb7db-ee85-48a7-8f41-e4603238bede RRID:CVCL_UI77 hTERT NF1 ipNF03.3 ["ipNF03.3"] cellLine Derived from a plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 c3fb3ba5-903b-4b31-a4f1-03462e18093f Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology a8e596b4-6067-4ac2-8d9c-7309f4b391ad null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c3fb3ba5-903b-4b31-a4f1-03462e18093f, null, null, 18, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, f21cb7db-ee85-48a7-8f41-e4603238bede, hTERT NF1 ipNF03.3, cellLine, RRID:CVCL_UI77, Male, Homo sapiens, null, null, ["ipNF03.3"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[The neurofibroma, a common feature of neurofibromatosis type 1 (NF1), is a benign peripheral nerve sheath tumor that contains predominantly Schwann cells (SC). There are reports that neurofibroma growth may be affected by hormonal changes, particularly in puberty and pregnancy, suggesting an influence by steroid hormones. This study examined the effects of estrogen and progesterone on proliferation and apoptosis in a panel of NF1 tumor xenografts. SC-enriched cultures derived from three human NF1 tumor types (dermal neurofibroma, plexiform neurofibroma, and malignant peripheral nerve sheath tumor (MPNST)) were xenografted in sciatic nerves of ovariectomized scid /Nf1-/+ mice. At the same time, mice were implanted with time-release pellets for systemic delivery of progesterone, estrogen or placebo. Proliferation and apoptosis by the xenografted SC were examined two months after implantation, by Ki67 immunolabeling and TUNEL. Estrogen was found to increase the growth of all three MPNST xenografts. Progesterone was associated with increased growth in two of the three MPNSTs, yet decreased growth of the other. Of the four dermal neurofibroma xenografts tested, estrogen caused a statistically significant growth increase in one, and progesterone did in another. Of the four plexiform neurofibroma SC xenografts, estrogen and progesterone significantly decreased growth in one of the xenografts, but not the other three. No relationship of patient age or gender to steroid response was observed. These findings indicate that human NF1 Schwann cells derived from some tumors show increased proliferation or decreased apoptosis in response to particular steroid hormones in a mouse xenograft model. This suggests that anti-estrogen or anti-progesterone therapies may be worth considering for specific NF1 neurofibromas and MPNSTs., null, null, ["Hua Li", "Xuelian Zhang", "Lauren Fishbein", "Frederick Kweh", "Martha Campbell-Thompson", "George Q Perrin", "David Muir", "Margaret Wallace"], CRL-2885, null, Cancer cell line, 39b4b78a-44f4-4c19-8b62-89ca471fb5d1, null, b72d99fd-ee1e-42ec-92b6-9f89e375cce1 RRID:CVCL_K280 sNF02.2 null cellLine MPNST tumor cell line from an NF1 patient. yes ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 7cd65ff8-953a-40ed-b318-b8562c646b11 Homo sapiens Male null b603b0a5-cc1d-48b3-aa03-e70a7d19eb1e CRL-2885 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ c21f4170-a611-4062-a455-26e74560a4d4 10.4161/cbt.10.8.12878 ["Hua Li", "Xuelian Zhang", "Lauren Fishbein", "Frederick Kweh", "Martha Campbell-Thompson", "George Q Perrin", "David Muir", "Margaret Wallace"] The neurofibroma, a common feature of neurofibromatosis type 1 (NF1), is a benign peripheral nerve sheath tumor that contains predominantly Schwann cells (SC). There are reports that neurofibroma growth may be affected by hormonal changes, particularly in puberty and pregnancy, suggesting an influence by steroid hormones. This study examined the effects of estrogen and progesterone on proliferation and apoptosis in a panel of NF1 tumor xenografts. SC-enriched cultures derived from three human NF1 tumor types (dermal neurofibroma, plexiform neurofibroma, and malignant peripheral nerve sheath tumor (MPNST)) were xenografted in sciatic nerves of ovariectomized scid /Nf1-/+ mice. At the same time, mice were implanted with time-release pellets for systemic delivery of progesterone, estrogen or placebo. Proliferation and apoptosis by the xenografted SC were examined two months after implantation, by Ki67 immunolabeling and TUNEL. Estrogen was found to increase the growth of all three MPNST xenografts. Progesterone was associated with increased growth in two of the three MPNSTs, yet decreased growth of the other. Of the four dermal neurofibroma xenografts tested, estrogen caused a statistically significant growth increase in one, and progesterone did in another. Of the four plexiform neurofibroma SC xenografts, estrogen and progesterone significantly decreased growth in one of the xenografts, but not the other three. No relationship of patient age or gender to steroid response was observed. These findings indicate that human NF1 Schwann cells derived from some tumors show increased proliferation or decreased apoptosis in response to particular steroid hormones in a mouse xenograft model. This suggests that anti-estrogen or anti-progesterone therapies may be worth considering for specific NF1 neurofibromas and MPNSTs. Cancer Biology & Therapy 39b4b78a-44f4-4c19-8b62-89ca471fb5d1 null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.4161/cbt.10.8.12878, 7cd65ff8-953a-40ed-b318-b8562c646b11, null, null, 28, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Cancer Biology & Therapy, ["malignant peripheral nerve sheath tumor"], yes, null, null, null, null, c21f4170-a611-4062-a455-26e74560a4d4, null, null, b72d99fd-ee1e-42ec-92b6-9f89e375cce1, sNF02.2, cellLine, RRID:CVCL_K280, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, b603b0a5-cc1d-48b3-aa03-e70a7d19eb1e, ATCC, https://www.atcc.org/]

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[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[null, null, null, null, 5PNF_TDiPSsv_PM_6, null, Induced pluripotent stem cell, 9d07a958-d8e8-447b-a337-d0be0ee206e5, null, Cutaneous neurofibroma-derived iPSC banked in the Barcelona node of the Spanish National Cell Bank., Neurofibromatosis type 1, null, fd6e6f02-bf0f-48db-b1ad-9696630bed4d, null, null, null, null, null, null, null, [cutaneous neurofibroma], unknown, null, null, null, null, null, null, null, 8ebe9be8-88df-4598-bd25-510de550ca5e, 5PNF_TDiPSsv_PM_6, cellLine, RRID:CVCL_UN14, Male, Homo sapiens, null, null, null, null, null, cutaneous neurofibroma, [unknown], 1821d1da-302e-4380-adb2-fc54809fbf6d, 4d9f670f-485c-439d-8be9-7418438950a7, BNLC, https://www.isciii.es/QueHacemos/Servicios/BIOBANCOS/BNLC/Paginas/LineasiPS.aspx]
[null, null, null, null, 5PNF_TDiPSsv_MM_4, null, Induced pluripotent stem cell, 9b21d7dd-38e0-41d9-8145-31381b5467ed, null, Cutaneous neurofibroma-derived iPSC banked in the Barcelona node of the Spanish National Cell Bank., Neurofibromatosis type 1, null, fd6e6f02-bf0f-48db-b1ad-9696630bed4d, null, null, null, null, null, null, null, [cutaneous neurofibroma], unknown, null, null, null, null, null, null, null, db6b3d0d-ee80-49ab-bb51-5672448e580b, 5PNF_TDiPSsv_MM_4, cellLine, RRID:CVCL_UN13, Male, Homo sapiens, null, null, null, null, null, cutaneous neurofibroma, [unknown], 1821d1da-302e-4380-adb2-fc54809fbf6d, bdd9bc5d-a4a1-4ce6-9ccc-0b7109136fd8, BNLC, https://www.isciii.es/QueHacemos/Servicios/BIOBANCOS/BNLC/Paginas/LineasiPS.aspx]
[null, null, null, null, 7PNF_SiPSrv_PM_12, null, Induced pluripotent stem cell, 0456c7c2-e5dc-4fb6-a424-5712162bcef2, null, Cutaneous neurofibroma-derived iPSC banked in the Barcelona node of the Spanish National Cell Bank., Neurofibromatosis type 1, null, 777cd68f-b90f-4b2d-87b0-586a8daa53ca, null, null, null, null, null, null, null, [cutaneous neurofibroma], unknown, null, null, null, null, null, null, null, 95997768-950e-4c8d-93bb-e6ed370e503f, 7PNF_SiPSrv_PM_12, cellLine, RRID:CVCL_UN16, Female, Homo sapiens, null, null, null, null, null, cutaneous neurofibroma, [unknown], 1821d1da-302e-4380-adb2-fc54809fbf6d, 090513e2-2af1-431d-9028-645b53b532d6, BNLC, https://www.isciii.es/QueHacemos/Servicios/BIOBANCOS/BNLC/Paginas/LineasiPS.aspx]
[null, null, null, null, 6PNF_SiPSrv_PM_2, null, Induced pluripotent stem cell, 9c2a480c-869d-4e49-b2e9-c168e351945a, null, Cutaneous neurofibroma-derived iPSC banked in the Barcelona node of the Spanish National Cell Bank., Neurofibromatosis type 1, null, 3ea81fd9-d3e9-46aa-8d04-89f3a9296ab1, null, null, null, null, null, null, null, [cutaneous neurofibroma], unknown, null, null, null, null, null, null, null, e4deb9a8-7929-44f2-974d-375771b8c681, 6PNF_SiPSrv_PM_2, cellLine, RRID:CVCL_UN15, Female, Homo sapiens, null, null, [6PNF SiPSrv_PM_2], null, null, cutaneous neurofibroma, [unknown], 1821d1da-302e-4380-adb2-fc54809fbf6d, d6acfa8c-7031-41f1-87c3-af31585bd984, BNLC, https://www.isciii.es/QueHacemos/Servicios/BIOBANCOS/BNLC/Paginas/LineasiPS.aspx]
[null, null, null, null, 3PNF_SiPSsv_MM_11, null, Induced pluripotent stem cell, 8d7c220e-8b67-4899-a19b-18ef275e6573, null, Cutaneous neurofibroma-derived iPSC banked in the Barcelona node of the Spanish National Cell Bank., Neurofibromatosis type 1, null, b9759537-b897-44c4-9657-01436af495d2, null, null, null, null, null, null, null, [cutaneous neurofibroma], unknown, null, null, null, null, null, null, null, 1cc872fc-6a95-4722-a598-bf60bb124b82, 3PNF_SiPSsv_MM_11, cellLine, RRID:CVCL_UN12, Female, Homo sapiens, null, null, null, null, null, cutaneous neurofibroma, [unknown], 1821d1da-302e-4380-adb2-fc54809fbf6d, 78d5db65-fb5b-4d10-b83f-5a3d8d2052e5, BNLC, https://www.isciii.es/QueHacemos/Servicios/BIOBANCOS/BNLC/Paginas/LineasiPS.aspx]
[null, null, null, null, 3PNF_FiPSsv_PM_2, null, Induced pluripotent stem cell, c83a38f4-5831-463c-8f0b-5fe95ee01665, null, Cutaneous neurofibroma-derived iPSC banked in the Barcelona node of the Spanish National Cell Bank., Neurofibromatosis type 1, null, b9759537-b897-44c4-9657-01436af495d2, null, null, null, null, null, null, null, [cutaneous neurofibroma], unknown, null, null, null, null, null, null, null, 7deb7765-38aa-4d8a-a3cb-bd4aefeb3a86, 3PNF_FiPSsv_PM_2, cellLine, RRID:CVCL_UN11, Female, Homo sapiens, null, null, null, null, null, cutaneous neurofibroma, [unknown], 1821d1da-302e-4380-adb2-fc54809fbf6d, 56d48f67-0615-4c9b-b92f-4134ed95e515, BNLC, https://www.isciii.es/QueHacemos/Servicios/BIOBANCOS/BNLC/Paginas/LineasiPS.aspx]

No results

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, [ipNF05.5 mixed clone, ipNF05.5 (mixed clone)], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, [ipNF95.11b C/T, ipNF95.11bC_T], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, [ipNF95.11b C, ipNF95.11bC], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, [ipnNF95.11c, ipnNF95.11C], null, null, Not Applicable, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, [ipNF05.5], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, [ipnNF09.4], null, null, Not Applicable, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, [ipNF04.4], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3389, null, Telomerase immortalized cell line, 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94, null, Derived from a plexiform neurofibroma growing on cranial nerve XII., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 108cfda9-d027-49cf-b950-d3e76097aa3c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, b44361f2-9021-4920-901a-b1a1f9143f97, hTERT NF1 ipNF95.6, cellLine, RRID:CVCL_UI70, Male, Homo sapiens, null, null, [ipNF95.6], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, e4426959-f253-4eac-8c68-3415f43104af, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, [ipNF06.2A], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, [sipnNF95.12B], null, null, Not Applicable, [unknown], null, null, null, null]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, [ipNF05.5 mixed clone, ipNF05.5 (mixed clone)], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, [ipNF95.11b C/T, ipNF95.11bC_T], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, [ipNF95.11b C, ipNF95.11bC], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, [ipnNF95.11c, ipnNF95.11C], null, null, Not Applicable, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, [ipNF05.5], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, [ipnNF09.4], null, null, Not Applicable, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, [ipNF04.4], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3389, null, Telomerase immortalized cell line, 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94, null, Derived from a plexiform neurofibroma growing on cranial nerve XII., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 108cfda9-d027-49cf-b950-d3e76097aa3c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, b44361f2-9021-4920-901a-b1a1f9143f97, hTERT NF1 ipNF95.6, cellLine, RRID:CVCL_UI70, Male, Homo sapiens, null, null, [ipNF95.6], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, e4426959-f253-4eac-8c68-3415f43104af, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, [ipNF06.2A], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, [sipnNF95.12B], null, null, Not Applicable, [unknown], null, null, null, null]

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[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, 844b598c-0171-4972-91c3-27aa21b45d52 RRID:CVCL_UI72 hTERT NF1 ipNF05.5 mixed clones ["ipNF05.5 mixed clone", "ipNF05.5 (mixed clone)"] cellLine Derived from a plexiform neurofibroma growing on a hand. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null 9a08aa2d-3d44-497f-9d49-12e9b30d82cf CRL-3387 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 71e33e5d-2392-4112-b284-1386842dd935 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 7, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, ["ipNF05.5 mixed clone", "ipNF05.5 (mixed clone)"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, ab60aae5-7860-4d1d-bb02-208e6631c78b RRID:CVCL_UI68 hTERT NF1 ipNF95.11b C/T ["ipNF95.11b C/T", "ipNF95.11bC_T"] cellLine Derived from a plexiform neurofibroma growing on a brachial plexus. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology c5037bab-6cce-49f6-91ab-2ac26753829b null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 8, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, ["ipNF95.11b C/T", "ipNF95.11bC_T"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, be2333d6-6716-4d13-947d-41f4198497a4 RRID:CVCL_UI67 hTERT NF1 ipNF95.11b C ["ipNF95.11b C", "ipNF95.11bC"] cellLine Derived from a plexiform neurofibroma growing on a brachial plexus. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null f67f594e-b2c0-4497-a5d9-4699b3b2d601 CRL-3390 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 36bd1db8-c1af-4825-a557-13d6276f7949 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 9, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, ["ipNF95.11b C", "ipNF95.11bC"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d RRID:CVCL_UI69 hTERT NF1 ipnNF95.11c ["ipnNF95.11c", "ipnNF95.11C"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. yes ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null 9787b08c-6ecf-4159-8918-928c800ad977 CRL-3391 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology bacda034-66c2-4d1d-b235-7258682342a2 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 10, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, ["ipnNF95.11c", "ipnNF95.11C"], null, null, Not Applicable, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f RRID:CVCL_UI71 hTERT NF1 ipNF05.5 ["ipNF05.5"] cellLine Derived from a plexiform neurofibroma growing on a hand. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null 071ef5b1-79cb-4a4d-ad61-900eea8f9be0 CRL-3388 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology d43864f1-277e-41dd-ae77-2d8b153b9be8 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 11, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, ["ipNF05.5"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304 RRID:CVCL_UI73 hTERT NF1 ipnNF09.4 ["ipnNF09.4"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. unknown ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 55e157c9-c4c1-40d8-9187-6e6866c3ab46 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 12, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, ["ipnNF09.4"], null, null, Not Applicable, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d RRID:CVCL_UI78 hTERT NF1 ipNF04.4 ["ipNF04.4"] cellLine Derived from a plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 c6a7d35b-580c-41ca-bf82-b5bfd5234289 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 4d77247b-d123-42cf-8110-d335d0a9c953 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, 13, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, ["ipNF04.4"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3389, null, Telomerase immortalized cell line, 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94, null, b44361f2-9021-4920-901a-b1a1f9143f97 RRID:CVCL_UI70 hTERT NF1 ipNF95.6 ["ipNF95.6"] cellLine Derived from a plexiform neurofibroma growing on cranial nerve XII. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 108cfda9-d027-49cf-b950-d3e76097aa3c Homo sapiens Male null e4426959-f253-4eac-8c68-3415f43104af CRL-3389 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on cranial nerve XII., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 108cfda9-d027-49cf-b950-d3e76097aa3c, null, null, 14, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, b44361f2-9021-4920-901a-b1a1f9143f97, hTERT NF1 ipNF95.6, cellLine, RRID:CVCL_UI70, Male, Homo sapiens, null, null, ["ipNF95.6"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, e4426959-f253-4eac-8c68-3415f43104af, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb RRID:CVCL_UI74 hTERT NF1 ipNF06.2A ["ipNF06.2A"] cellLine Derived from a pleural plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 10a5e6f2-d331-4b5e-9e43-888cca69d5a9 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology faff6884-47dd-4bbf-ab57-f420d87d456e null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, 15, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, ["ipNF06.2A"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, 98beda5b-9b28-4119-829a-2a0219d77af7 RRID:CVCL_UI75 hTERT NF1 sipnNF95.12B ["sipnNF95.12B"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. unknown ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4e75c936-aa0d-4689-a0fd-d41b0072bc5c Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 428d6875-ad27-4ed5-ac53-39cb73697ca6 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, 16, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, ["sipnNF95.12B"], null, null, Not Applicable, ["unknown"], null, null, null, null]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[A novel cell line, YST-1, was established from an epithelioid malignant schwannoma (EMS) that occurred in the upper arm of an 8-year-old girl. YST-1 cells were polygonal and stellate in shape, contained abundant free ribosomes, mitochondria, lysosomes and rough-surfaced endoplasmic reticulum, and grew stably with a population doubling time of 40 h. Immunohistochemically, vimentin, S100 protein and S100 protein beta subunit were positive in the cytoplasm. The xeno-transplanted tumor in nude mice was composed of cells with an epithelioid arrangement similar to the original tumor. The borders of the tumor cells were connected intimately without desmosomal junctions, and there were abundant organelles in the cytoplasm. YST-1 cells were considered to be of value for studying the nature and histogenesis of EMS., null, null, [Y Nagashima, Y Ohaki, Y Tanaka, K Sumino, T Funabiki, T Okuyama, S Watanabe, M Umeda, K Misugi], null, null, Cancer cell line, 1def3485-d42c-4154-b06f-589d02e1ad86, null, Schwannoma cell line, potentially mischaracterized in some cases as a sporadic MPNST cell line., No known disease, 10.1007/BF02899420, b3964ac1-90da-4af6-b6d8-0dbe343167c2, null, null, null, null, null, null, Virchows Archiv. B, Cell Pathology Including Molecular Pathology, [schwannoma], unknown, null, null, null, null, 2a2cb57f-86ca-42cf-aad2-930aae16339b, Asian, null, 617cb183-3377-4473-8ad8-2f6472bce1fa, YST-1, cellLine, RRID:CVCL_5192, Female, Homo sapiens, null, null, [YST1], null, null, schwannoma, [unknown], null, null, null, null]
[Neurofibromatosis type 1 (NF1) is characterized by the formation of neurofibromas, benign tumors of the peripheral nerve consisting essentially of Schwann cells, which can sometimes turn malignant to form neurofibrosarcomas. The mechanism of progression toward a malignant phenotype remains largely unknown. In this report, we show that platelet-derived growth factor (PDGF) BB, and to a lesser extent fibroblast growth factor 2, are mitogenic for two neurofibrosarcoma-derived Schwann cell lines, but not for a Schwann cell line derived from a schwannoma (from a non-NF1 patient) or for transformed rat Schwann cells. Levels of expression of both PDGF receptor alpha and beta are significantly increased in the two neurofibrosarcoma-derived cell lines compared to the non-NF1 Schwann cell lines. The level of tyrosyl-phosphorylated PDGF receptor beta is strongly increased upon stimulation by PDGF BB. In comparison, only modest levels of tyrosyl-phosphorylated PDGF receptor alpha are observed, upon stimulation by PDGF AA or PDGF BB. Accordingly, PDGF AA is only a weak mitogen for the neurofibrosarcoma-derived cells by comparison to PDGF BB. These results indicate that the mitogenic effect of PDGF BB for the neurofibrosarcoma-derived Schwann cell lines is primarily transduced by PDGF receptor beta. Neu differentiation factor beta, a potent mitogen for normal Schwann cells, was unable to stimulate proliferation of the transformed Schwann cell lines, due to a dramatic down-regulation of the erbB3 receptor. Therefore, aberrant expression of growth factor receptors by Schwann cells, such as the PDGF receptors, could represent an important step in the process leading to Schwann cell hyperplasia in NF1., null, null, [A Badache, G H De Vries], null, null, Cancer cell line, 394dd9ac-6a36-4515-bcc3-4012ba60c86d, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1002/(SICI)1097-4652(199811)177:2<334::AID-JCP15>3.0.CO;2-9, 18f6f681-54bc-431c-a447-3f3d4eae8229, null, null, null, null, null, null, Journal of Cellular Physiology, [malignant peripheral nerve sheath tumor], unknown, null, null, null, null, b9fae169-947b-413a-8d99-3dd094355943, null, null, 6419dd0d-1937-4ecf-bf01-876632ae0f54, T265, cellLine, RRID:CVCL_S805, Unknown, Homo sapiens, null, null, [T265-2c, T265-2C, T265p21], null, null, malignant peripheral nerve sheath tumor, [unknown], null, null, null, null]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[A novel cell line, YST-1, was established from an epithelioid malignant schwannoma (EMS) that occurred in the upper arm of an 8-year-old girl. YST-1 cells were polygonal and stellate in shape, contained abundant free ribosomes, mitochondria, lysosomes and rough-surfaced endoplasmic reticulum, and grew stably with a population doubling time of 40 h. Immunohistochemically, vimentin, S100 protein and S100 protein beta subunit were positive in the cytoplasm. The xeno-transplanted tumor in nude mice was composed of cells with an epithelioid arrangement similar to the original tumor. The borders of the tumor cells were connected intimately without desmosomal junctions, and there were abundant organelles in the cytoplasm. YST-1 cells were considered to be of value for studying the nature and histogenesis of EMS., null, null, [Y Nagashima, Y Ohaki, Y Tanaka, K Sumino, T Funabiki, T Okuyama, S Watanabe, M Umeda, K Misugi], null, null, Cancer cell line, 1def3485-d42c-4154-b06f-589d02e1ad86, null, Schwannoma cell line, potentially mischaracterized in some cases as a sporadic MPNST cell line., No known disease, 10.1007/BF02899420, b3964ac1-90da-4af6-b6d8-0dbe343167c2, null, null, null, null, null, null, Virchows Archiv. B, Cell Pathology Including Molecular Pathology, [schwannoma], unknown, null, null, null, null, 2a2cb57f-86ca-42cf-aad2-930aae16339b, Asian, null, 617cb183-3377-4473-8ad8-2f6472bce1fa, YST-1, cellLine, RRID:CVCL_5192, Female, Homo sapiens, null, null, [YST1], null, null, schwannoma, [unknown], null, null, null, null]
[Tumor cell lines derived from malignant schwannomas removed from patients with neurofibromatosis type 1 (NF1) have been examined for the level of expression of NF1 protein. All three NF1 lines examined expressed lower levels of NF1 protein than control cells, and the level in one line was barely detectable. The tumor lines expressed normal levels of p120GAP and p21ras. Although the p21ras proteins isolated from the tumor cells had normal (nonmutant) biochemical properties in vitro, they displayed elevated levels of bound GTP in vivo. The level of total cellular GAP-like activity was reduced in extracts from the tumor line that expresses very little NF1 protein. Introduction of the catalytic region of GAP into this line resulted in morphological reversion and lower in vivo GTP binding by endogenous p21ras. These data implicate NF1 protein as a tumor suppressor gene product that negatively regulates p21ras and define a "positive" growth role for ras activity in NF1 malignancies., null, null, [J E DeClue, A G Papageorge, J A Fletcher, S R Diehl, N Ratner, W C Vass, D R Lowy], null, null, Cancer cell line, d0a04c27-a642-45dd-b00c-7b483d18c1f8, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1016/0092-8674(92)90407-4, 06ddf02a-9d00-428c-9e6d-fab9d2e21dc4, null, null, null, 1821d1da-302e-4380-adb2-fc54809fbf6d, Nancy Ratner, https://www.cincinnatichildrens.org/research/divisions/e/ex-hem/labs/ratner, Cell, [malignant peripheral nerve sheath tumor], unknown, null, null, null, null, 02662017-48d1-4633-8afd-fc488a972e74, null, null, 0f404e70-2acf-4877-bcd5-6da81d9fa41e, 90-8, cellLine, RRID:CVCL_1B47, Female, Homo sapiens, null, null, [MPNST 90-8TL, 90-8TL, NF90-8, NF190-8], null, null, malignant peripheral nerve sheath tumor, [unknown], null, null, null, null]
[Neurofibromatosis type 1 (NF1) is characterized by the formation of neurofibromas, benign tumors of the peripheral nerve consisting essentially of Schwann cells, which can sometimes turn malignant to form neurofibrosarcomas. The mechanism of progression toward a malignant phenotype remains largely unknown. In this report, we show that platelet-derived growth factor (PDGF) BB, and to a lesser extent fibroblast growth factor 2, are mitogenic for two neurofibrosarcoma-derived Schwann cell lines, but not for a Schwann cell line derived from a schwannoma (from a non-NF1 patient) or for transformed rat Schwann cells. Levels of expression of both PDGF receptor alpha and beta are significantly increased in the two neurofibrosarcoma-derived cell lines compared to the non-NF1 Schwann cell lines. The level of tyrosyl-phosphorylated PDGF receptor beta is strongly increased upon stimulation by PDGF BB. In comparison, only modest levels of tyrosyl-phosphorylated PDGF receptor alpha are observed, upon stimulation by PDGF AA or PDGF BB. Accordingly, PDGF AA is only a weak mitogen for the neurofibrosarcoma-derived cells by comparison to PDGF BB. These results indicate that the mitogenic effect of PDGF BB for the neurofibrosarcoma-derived Schwann cell lines is primarily transduced by PDGF receptor beta. Neu differentiation factor beta, a potent mitogen for normal Schwann cells, was unable to stimulate proliferation of the transformed Schwann cell lines, due to a dramatic down-regulation of the erbB3 receptor. Therefore, aberrant expression of growth factor receptors by Schwann cells, such as the PDGF receptors, could represent an important step in the process leading to Schwann cell hyperplasia in NF1., null, null, [A Badache, G H De Vries], null, null, Cancer cell line, 394dd9ac-6a36-4515-bcc3-4012ba60c86d, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1002/(SICI)1097-4652(199811)177:2<334::AID-JCP15>3.0.CO;2-9, 18f6f681-54bc-431c-a447-3f3d4eae8229, null, null, null, null, null, null, Journal of Cellular Physiology, [malignant peripheral nerve sheath tumor], unknown, null, null, null, null, b9fae169-947b-413a-8d99-3dd094355943, null, null, 6419dd0d-1937-4ecf-bf01-876632ae0f54, T265, cellLine, RRID:CVCL_S805, Unknown, Homo sapiens, null, null, [T265-2c, T265-2C, T265p21], null, null, malignant peripheral nerve sheath tumor, [unknown], null, null, null, null]

[ABSTRACT, ANIMALMODELID, ANIMALSTATE, AUTHORS, CATALOGNUMBER, CATALOGNUMBERURL, CELLLINECATEGORY, CELLLINEID, CONTAMINATEDMISIDENTIFIED, CONTENT_TEXT, DESCRIPTION, DISEASE, DOI, DONORID, GENERATION, GENETICBACKGROUND, ID, INSTITUTION, INVESTIGATORID, INVESTIGATORNAME, INVESTIGATORWEBSITE, JOURNAL, MODELOFMANIFESTATION, MTAREQUIRED, ORCID, ORGAN, ORIGINYEAR, POPULATIONDOUBLINGTIME, PUBLICATIONID, RACE, RESISTANCE, RESOURCEID, RESOURCENAME, RESOURCETYPE, RRID, SEX, SPECIES, STRAINNOMENCLATURE, STRPROFILE, SYNONYMS, TISSUE, TRANSPLANTATIONTYPE, TUMORTYPE, USAGERESTRICTIONS, VENDORID, VENDORITEMID, VENDORNAME, WEBSITE]
[A novel cell line, YST-1, was established from an epithelioid malignant schwannoma (EMS) that occurred in the upper arm of an 8-year-old girl. YST-1 cells were polygonal and stellate in shape, contained abundant free ribosomes, mitochondria, lysosomes and rough-surfaced endoplasmic reticulum, and grew stably with a population doubling time of 40 h. Immunohistochemically, vimentin, S100 protein and S100 protein beta subunit were positive in the cytoplasm. The xeno-transplanted tumor in nude mice was composed of cells with an epithelioid arrangement similar to the original tumor. The borders of the tumor cells were connected intimately without desmosomal junctions, and there were abundant organelles in the cytoplasm. YST-1 cells were considered to be of value for studying the nature and histogenesis of EMS., null, null, ["Y Nagashima", "Y Ohaki", "Y Tanaka", "K Sumino", "T Funabiki", "T Okuyama", "S Watanabe", "M Umeda", "K Misugi"], null, null, Cancer cell line, 1def3485-d42c-4154-b06f-589d02e1ad86, null, 617cb183-3377-4473-8ad8-2f6472bce1fa RRID:CVCL_5192 YST-1 ["YST1"] cellLine Schwannoma cell line, potentially mischaracterized in some cases as a sporadic MPNST cell line. unknown ["unknown"] null null null null null null ["schwannoma"] No known disease b3964ac1-90da-4af6-b6d8-0dbe343167c2 Homo sapiens Female Asian null null null null null null null null null null null 2a2cb57f-86ca-42cf-aad2-930aae16339b 10.1007/BF02899420 ["Y Nagashima", "Y Ohaki", "Y Tanaka", "K Sumino", "T Funabiki", "T Okuyama", "S Watanabe", "M Umeda", "K Misugi"] A novel cell line, YST-1, was established from an epithelioid malignant schwannoma (EMS) that occurred in the upper arm of an 8-year-old girl. YST-1 cells were polygonal and stellate in shape, contained abundant free ribosomes, mitochondria, lysosomes and rough-surfaced endoplasmic reticulum, and grew stably with a population doubling time of 40 h. Immunohistochemically, vimentin, S100 protein and S100 protein beta subunit were positive in the cytoplasm. The xeno-transplanted tumor in nude mice was composed of cells with an epithelioid arrangement similar to the original tumor. The borders of the tumor cells were connected intimately without desmosomal junctions, and there were abundant organelles in the cytoplasm. YST-1 cells were considered to be of value for studying the nature and histogenesis of EMS. Virchows Archiv. B, Cell Pathology Including Molecular Pathology 1def3485-d42c-4154-b06f-589d02e1ad86 null null Cancer cell line null null null null null schwannoma , Schwannoma cell line, potentially mischaracterized in some cases as a sporadic MPNST cell line., No known disease, 10.1007/BF02899420, b3964ac1-90da-4af6-b6d8-0dbe343167c2, null, null, 46, null, null, null, null, Virchows Archiv. B, Cell Pathology Including Molecular Pathology, ["schwannoma"], unknown, null, null, null, null, 2a2cb57f-86ca-42cf-aad2-930aae16339b, Asian, null, 617cb183-3377-4473-8ad8-2f6472bce1fa, YST-1, cellLine, RRID:CVCL_5192, Female, Homo sapiens, null, null, ["YST1"], null, null, schwannoma, ["unknown"], null, null, null, null]
[Neurofibromatosis type 1 (NF1) is characterized by the formation of neurofibromas, benign tumors of the peripheral nerve consisting essentially of Schwann cells, which can sometimes turn malignant to form neurofibrosarcomas. The mechanism of progression toward a malignant phenotype remains largely unknown. In this report, we show that platelet-derived growth factor (PDGF) BB, and to a lesser extent fibroblast growth factor 2, are mitogenic for two neurofibrosarcoma-derived Schwann cell lines, but not for a Schwann cell line derived from a schwannoma (from a non-NF1 patient) or for transformed rat Schwann cells. Levels of expression of both PDGF receptor alpha and beta are significantly increased in the two neurofibrosarcoma-derived cell lines compared to the non-NF1 Schwann cell lines. The level of tyrosyl-phosphorylated PDGF receptor beta is strongly increased upon stimulation by PDGF BB. In comparison, only modest levels of tyrosyl-phosphorylated PDGF receptor alpha are observed, upon stimulation by PDGF AA or PDGF BB. Accordingly, PDGF AA is only a weak mitogen for the neurofibrosarcoma-derived cells by comparison to PDGF BB. These results indicate that the mitogenic effect of PDGF BB for the neurofibrosarcoma-derived Schwann cell lines is primarily transduced by PDGF receptor beta. Neu differentiation factor beta, a potent mitogen for normal Schwann cells, was unable to stimulate proliferation of the transformed Schwann cell lines, due to a dramatic down-regulation of the erbB3 receptor. Therefore, aberrant expression of growth factor receptors by Schwann cells, such as the PDGF receptors, could represent an important step in the process leading to Schwann cell hyperplasia in NF1., null, null, ["A Badache", "G H De Vries"], null, null, Cancer cell line, 394dd9ac-6a36-4515-bcc3-4012ba60c86d, null, 6419dd0d-1937-4ecf-bf01-876632ae0f54 RRID:CVCL_S805 T265 ["T265-2c", "T265-2C", "T265p21"] cellLine MPNST tumor cell line from an NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 18f6f681-54bc-431c-a447-3f3d4eae8229 Homo sapiens Unknown null null null null null null null null null null null null b9fae169-947b-413a-8d99-3dd094355943 10.1002/(SICI)1097-4652(199811)177:2<334::AID-JCP15>3.0.CO;2-9 ["A Badache", "G H De Vries"] Neurofibromatosis type 1 (NF1) is characterized by the formation of neurofibromas, benign tumors of the peripheral nerve consisting essentially of Schwann cells, which can sometimes turn malignant to form neurofibrosarcomas. The mechanism of progression toward a malignant phenotype remains largely unknown. In this report, we show that platelet-derived growth factor (PDGF) BB, and to a lesser extent fibroblast growth factor 2, are mitogenic for two neurofibrosarcoma-derived Schwann cell lines, but not for a Schwann cell line derived from a schwannoma (from a non-NF1 patient) or for transformed rat Schwann cells. Levels of expression of both PDGF receptor alpha and beta are significantly increased in the two neurofibrosarcoma-derived cell lines compared to the non-NF1 Schwann cell lines. The level of tyrosyl-phosphorylated PDGF receptor beta is strongly increased upon stimulation by PDGF BB. In comparison, only modest levels of tyrosyl-phosphorylated PDGF receptor alpha are observed, upon stimulation by PDGF AA or PDGF BB. Accordingly, PDGF AA is only a weak mitogen for the neurofibrosarcoma-derived cells by comparison to PDGF BB. These results indicate that the mitogenic effect of PDGF BB for the neurofibrosarcoma-derived Schwann cell lines is primarily transduced by PDGF receptor beta. Neu differentiation factor beta, a potent mitogen for normal Schwann cells, was unable to stimulate proliferation of the transformed Schwann cell lines, due to a dramatic down-regulation of the erbB3 receptor. Therefore, aberrant expression of growth factor receptors by Schwann cells, such as the PDGF receptors, could represent an important step in the process leading to Schwann cell hyperplasia in NF1. Journal of Cellular Physiology 394dd9ac-6a36-4515-bcc3-4012ba60c86d null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1002/(SICI)1097-4652(199811)177:2<334::AID-JCP15>3.0.CO;2-9, 18f6f681-54bc-431c-a447-3f3d4eae8229, null, null, 51, null, null, null, null, Journal of Cellular Physiology, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, b9fae169-947b-413a-8d99-3dd094355943, null, null, 6419dd0d-1937-4ecf-bf01-876632ae0f54, T265, cellLine, RRID:CVCL_S805, Unknown, Homo sapiens, null, null, ["T265-2c", "T265-2C", "T265p21"], null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Human neurofibromatosis type 1 is a dominant disease caused by the inheritance of a mutant allele of the NF1 gene. In order to study NF1 function, we have constructed a mouse strain carrying a germline mutation in the murine homologue. Heterozygous animals do not exhibit the classical symptoms of the human disease, but are highly predisposed to the formation of various tumour types, notably phaeochomocytoma, a tumour of the neural crest-derived adrenal medulla, and myeloid leukaemia, both of which occur with increased frequency in human NF1 patients. The wild-type Nf1 allele is lost in approximately half of the tumours from heterozygous animals. In addition, homozygosity for the Nf1 mutation leads to abnormal cardiac development and mid-gestational embryonic lethality., c9ccb5f0-587d-4c2c-ba8b-184013b41d44, mouse cells, [T Jacks, T S Shih, E M Schmitt, R T Bronson, A Bernards, R A Weinberg], JAX_008192, https://www.jax.org/strain/008192, null, null, null, [From JAX]: Heterozygous animals do not exhibit the classical symptoms of Human neurofibromatosis type 1, but are highly predisposed to the formation of various tumor types, notably phaeochromocytoma, a tumor of the neural crest-derived adrenal medulla, and myeloid leukemia. Homozygosity leads to abnormal cardiac development and mid-gestational embryonic lethality. This strain may be useful in studies of cancer and developmental biology., Neurofibromatosis type 1, 10.1038/ng0794-353, 7b50b518-1d02-4186-a701-733abd204b78, null, C57BL/6, Massachusetts Institute of Technology, bba58be3-c41e-4d53-bf4e-7cd9dbbecc0e, Tyler Jacks, null, Nature Genetics, [pheochromocytoma, acute myeloid leukemia, tumor of the neural crest-derived adrenal medulla], yes, null, null, null, null, 09f4d299-2d0c-4831-82a5-eae2ef3229d2, null, null, 45638793-f2d0-4c40-8b1c-be1f1b0c0f93, B6.129S2-Nf1tm1Tyj/J, animalModel, RRID:IMSR_JAX:008192, null, Mus musculus, null, B6.129S2-Nf1tm1Tyj/J, null, null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, 81042b9d-3d66-45c8-9c7d-8b0c90bb4b6c, The Jackson Laboratory, https://www.jax.org]
[null, null, null, null, GM11602, null, Transformed cell line, a76cad86-d2a2-402d-bb06-5ee13a8cdcec, null, Leukemia cell line derived from B-lymphocytes from an NF1 patient., Neurofibromatosis type 1, null, 49b6cb1f-7231-4208-a012-41c9bffff993, null, null, null, null, null, null, null, [acute lymphocytic leukemia], unknown, null, null, null, null, null, White, null, f354ec1a-0305-4e6c-897a-10e4fba10a28, GM11602, cellLine, RRID:CVCL_AA02, Female, Homo sapiens, null, null, null, null, null, acute lymphocytic leukemia, [unknown], 6c22c677-407b-463c-b53c-6dfa3c50e681, 56da4429-da43-44c8-862b-8a70955091df, Corielle Institute, https://www.coriell.org]
[null, null, null, null, GM11601, null, Transformed cell line, 58d36942-062c-4db1-91f9-ee8cda50f4fc, null, Leukemia cell line derived from B-lymphocytes from an NF1 patient., Neurofibromatosis type 1, null, 0460814f-4395-46bd-8acf-f1ded8afde9d, null, null, null, null, null, null, null, [acute lymphocytic leukemia], unknown, null, null, null, null, null, White, null, a2f62c57-a1b8-4867-9c91-04406f261cfa, GM11601, cellLine, RRID:CVCL_AA01, Male, Homo sapiens, null, null, null, null, null, acute lymphocytic leukemia, [unknown], 6c22c677-407b-463c-b53c-6dfa3c50e681, 495b8f81-36bc-4273-8e78-27ed87ef48c1, Corielle Institute, https://www.coriell.org]
[null, null, null, null, JCRB3015, null, Finite cell line, 50ca52b3-8448-4e82-a9ee-148d6cda19f1, null, Cell line from an NF1 patient; unclear if derived from tumor or non-tumor tissue., Neurofibromatosis type 1, null, f860ef0a-2501-4fa1-b0be-f638bde25c6d, null, null, null, null, null, null, null, [general NF1 deficiency], unknown, null, null, null, null, null, Asian, null, 1a8a362e-e3be-4bc3-be7e-d0a0865b9c31, NF1, cellLine, RRID:CVCL_JG80, Male, Homo sapiens, null, null, null, null, null, Not Applicable, [unknown], 247530ea-aba9-4560-9c67-39f6f9de532f, 111541ac-94a0-4190-babc-cf5be4bbd1ab, JCRB Cell Bank, https://cellbank.nibiohn.go.jp]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, [ipnNF95.11c, ipnNF95.11C], null, null, Not Applicable, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, [ipnNF09.4], null, null, Not Applicable, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, [sipnNF95.12B], null, null, Not Applicable, [unknown], null, null, null, null]
[Malignant soft-tissue tumors often present substantial diagnostic challenges. Chromosome aberrations that might be diagnostic have been identified in some types of soft-tissue tumors, but the overall frequency and diagnostic relevance of these aberrations have not been established. We attempted to determine the karyotypes of a series of 62 consecutive, unselected malignant spindle-cell or small round-cell soft-tissue tumors (from 46 adults and 16 children) after direct harvesting of cells or short-term culture. All tumors were examined independently by immunohistochemical staining in addition to routine light-microscopical evaluation, and all but two tumors were examined by electron microscopy. Metaphases were obtained from 61 of the 62 tumors, and clonal chromosome aberrations were identified in 55 (89 percent). In the six tumors that yielded metaphases but lacked apparent clonal aberrations, the normal metaphases were found to originate from non-neoplastic stromal elements within the tumor specimens. Thus, all tumors in which karyotyping was successful contained clonal chromosome aberrations. Forty of 62 tumors (65 percent) contained clonal chromosome aberrations that either suggested or confirmed a specific diagnosis; in 15 of these tumors (24 percent of all tumors), the aberrations were important in establishing the final diagnosis. Cytogenetic analyses were particularly informative about small round-cell tumors from children: 8 of 14 round-cell tumors contained diagnostically important chromosome aberrations. Using the combined approaches of light and electron microscopy, immunohistochemistry, and cytogenetics, we established an unambiguous diagnosis for 60 of 62 tumors. Cytogenetic analyses reveal clonal chromosome aberrations in virtually all malignant soft-tissue tumors. These clonal chromosome aberrations, particularly in small round-cell tumors in children, often have diagnostic relevance., null, null, [J A Fletcher, H P Kozakewich, F A Hoffer, J M Lage, N Weidner, R Tepper, G S Pinkus, C C Morton, J M Corson], null, null, Cancer cell line, ce2107ee-f903-4ed9-a6d7-3c91de703f4e, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1056/NEJM199102143240702, f926219f-4a17-45cc-a41a-aee6abb4c761, null, null, null, null, null, null, The New England Journal of Medicine, [malignant peripheral nerve sheath tumor], unknown, null, null, null, null, 0235bd13-fd94-4b95-9e98-19c1c4d9afc2, null, null, 202c110b-a5f1-49ab-acdc-e6e33a1c29bb, ST88-14, cellLine, RRID:CVCL_8916, Male, Homo sapiens, null, null, [ST88.14, ST 88-14, ST-8814, ST8814, 88-14, NF188-14], null, null, malignant peripheral nerve sheath tumor, [unknown], null, null, null, null]
[Malignant peripheral nerve sheath tumors (MPNST) are the most aggressive cancers associated with neurofibromatosis type 1 (NF1). Here we report a practical and reproducible model of intraneural NF1 MPNST, by orthotopic xenograft of an immortal human NF1 tumor-derived Schwann cell line into the sciatic nerves of female scid mice. Intraneural injection of the cell line sNF96.2 consistently produced MPNST-like tumors that were highly cellular and showed extensive intraneural growth. These xenografts had a high proliferative index, were angiogenic, had significant mast cell infiltration and rapidly dominated the host nerve. The histopathology of engrafted intraneural tumors was consistent with that of human NF1 MPNST. Xenograft tumors were readily examined by magnetic resonance imaging, which also was used to assess tumor vascularity. In addition, the intraneural proliferation of sNF96.2 cell tumors was decreased in ovariectomized mice, while replacement of estrogen or progesterone restored tumor cell proliferation. This suggests a potential role for steroid hormones in supporting tumor cell growth of this MPNST cell line in vivo. The controlled orthotopic implantation of sNF96.2 cells provides for the precise initiation of intraneural MPNST-like tumors in a model system suitable for therapeutic interventions, including inhibitors of angiogenesis and further study of steroid hormone effects on tumor cell growth., null, null, [George Q Perrin, Hua Li, Lauren Fishbein, Susanne A Thomson, Min S Hwang, Mark T Scarborough, Anthony T Yachnis, Margaret R Wallace, Thomas H Mareci, David Muir], CRL-2884, null, Cancer cell line, ca79ff41-46a9-4a9f-9a12-55810fd9267b, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1038/labinvest.3700675, 63858b37-331f-430d-bcfa-01f6836a6003, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [malignant peripheral nerve sheath tumor], yes, null, null, null, null, bdd70b67-9ef6-4809-ba5b-51bb39cf63a0, null, null, c9a87975-378b-4930-8fda-e5896c42c86c, sNF96.2, cellLine, RRID:CVCL_K281, Male, Homo sapiens, null, null, [SNF96.2, sNF96-2], null, null, malignant peripheral nerve sheath tumor, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 26dd6c4b-f37a-4376-bb29-c78ee6924034, ATCC, https://www.atcc.org/]
[The neurofibroma, a common feature of neurofibromatosis type 1 (NF1), is a benign peripheral nerve sheath tumor that contains predominantly Schwann cells (SC). There are reports that neurofibroma growth may be affected by hormonal changes, particularly in puberty and pregnancy, suggesting an influence by steroid hormones. This study examined the effects of estrogen and progesterone on proliferation and apoptosis in a panel of NF1 tumor xenografts. SC-enriched cultures derived from three human NF1 tumor types (dermal neurofibroma, plexiform neurofibroma, and malignant peripheral nerve sheath tumor (MPNST)) were xenografted in sciatic nerves of ovariectomized scid /Nf1-/+ mice. At the same time, mice were implanted with time-release pellets for systemic delivery of progesterone, estrogen or placebo. Proliferation and apoptosis by the xenografted SC were examined two months after implantation, by Ki67 immunolabeling and TUNEL. Estrogen was found to increase the growth of all three MPNST xenografts. Progesterone was associated with increased growth in two of the three MPNSTs, yet decreased growth of the other. Of the four dermal neurofibroma xenografts tested, estrogen caused a statistically significant growth increase in one, and progesterone did in another. Of the four plexiform neurofibroma SC xenografts, estrogen and progesterone significantly decreased growth in one of the xenografts, but not the other three. No relationship of patient age or gender to steroid response was observed. These findings indicate that human NF1 Schwann cells derived from some tumors show increased proliferation or decreased apoptosis in response to particular steroid hormones in a mouse xenograft model. This suggests that anti-estrogen or anti-progesterone therapies may be worth considering for specific NF1 neurofibromas and MPNSTs., null, null, [Hua Li, Xuelian Zhang, Lauren Fishbein, Frederick Kweh, Martha Campbell-Thompson, George Q Perrin, David Muir, Margaret Wallace], CRL-2885, null, Cancer cell line, 39b4b78a-44f4-4c19-8b62-89ca471fb5d1, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.4161/cbt.10.8.12878, 7cd65ff8-953a-40ed-b318-b8562c646b11, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Cancer Biology & Therapy, [malignant peripheral nerve sheath tumor], yes, null, null, null, null, c21f4170-a611-4062-a455-26e74560a4d4, null, null, b72d99fd-ee1e-42ec-92b6-9f89e375cce1, sNF02.2, cellLine, RRID:CVCL_K280, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, b603b0a5-cc1d-48b3-aa03-e70a7d19eb1e, ATCC, https://www.atcc.org/]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Human neurofibromatosis type 1 is a dominant disease caused by the inheritance of a mutant allele of the NF1 gene. In order to study NF1 function, we have constructed a mouse strain carrying a germline mutation in the murine homologue. Heterozygous animals do not exhibit the classical symptoms of the human disease, but are highly predisposed to the formation of various tumour types, notably phaeochomocytoma, a tumour of the neural crest-derived adrenal medulla, and myeloid leukaemia, both of which occur with increased frequency in human NF1 patients. The wild-type Nf1 allele is lost in approximately half of the tumours from heterozygous animals. In addition, homozygosity for the Nf1 mutation leads to abnormal cardiac development and mid-gestational embryonic lethality., c9ccb5f0-587d-4c2c-ba8b-184013b41d44, mouse cells, [T Jacks, T S Shih, E M Schmitt, R T Bronson, A Bernards, R A Weinberg], JAX_008192, https://www.jax.org/strain/008192, null, null, null, [From JAX]: Heterozygous animals do not exhibit the classical symptoms of Human neurofibromatosis type 1, but are highly predisposed to the formation of various tumor types, notably phaeochromocytoma, a tumor of the neural crest-derived adrenal medulla, and myeloid leukemia. Homozygosity leads to abnormal cardiac development and mid-gestational embryonic lethality. This strain may be useful in studies of cancer and developmental biology., Neurofibromatosis type 1, 10.1038/ng0794-353, 7b50b518-1d02-4186-a701-733abd204b78, null, C57BL/6, Massachusetts Institute of Technology, bba58be3-c41e-4d53-bf4e-7cd9dbbecc0e, Tyler Jacks, null, Nature Genetics, [pheochromocytoma, acute myeloid leukemia, tumor of the neural crest-derived adrenal medulla], yes, null, null, null, null, 09f4d299-2d0c-4831-82a5-eae2ef3229d2, null, null, 45638793-f2d0-4c40-8b1c-be1f1b0c0f93, B6.129S2-Nf1tm1Tyj/J, animalModel, RRID:IMSR_JAX:008192, null, Mus musculus, null, B6.129S2-Nf1tm1Tyj/J, null, null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, 81042b9d-3d66-45c8-9c7d-8b0c90bb4b6c, The Jackson Laboratory, https://www.jax.org]
[null, null, null, null, GM11602, null, Transformed cell line, a76cad86-d2a2-402d-bb06-5ee13a8cdcec, null, Leukemia cell line derived from B-lymphocytes from an NF1 patient., Neurofibromatosis type 1, null, 49b6cb1f-7231-4208-a012-41c9bffff993, null, null, null, null, null, null, null, [acute lymphocytic leukemia], unknown, null, null, null, null, null, White, null, f354ec1a-0305-4e6c-897a-10e4fba10a28, GM11602, cellLine, RRID:CVCL_AA02, Female, Homo sapiens, null, null, null, null, null, acute lymphocytic leukemia, [unknown], 6c22c677-407b-463c-b53c-6dfa3c50e681, 56da4429-da43-44c8-862b-8a70955091df, Corielle Institute, https://www.coriell.org]
[null, null, null, null, GM11601, null, Transformed cell line, 58d36942-062c-4db1-91f9-ee8cda50f4fc, null, Leukemia cell line derived from B-lymphocytes from an NF1 patient., Neurofibromatosis type 1, null, 0460814f-4395-46bd-8acf-f1ded8afde9d, null, null, null, null, null, null, null, [acute lymphocytic leukemia], unknown, null, null, null, null, null, White, null, a2f62c57-a1b8-4867-9c91-04406f261cfa, GM11601, cellLine, RRID:CVCL_AA01, Male, Homo sapiens, null, null, null, null, null, acute lymphocytic leukemia, [unknown], 6c22c677-407b-463c-b53c-6dfa3c50e681, 495b8f81-36bc-4273-8e78-27ed87ef48c1, Corielle Institute, https://www.coriell.org]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, [ipnNF95.11c, ipnNF95.11C], null, null, Not Applicable, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, [ipnNF09.4], null, null, Not Applicable, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, [sipnNF95.12B], null, null, Not Applicable, [unknown], null, null, null, null]
[null, null, null, null, JCRB3015, null, Finite cell line, 50ca52b3-8448-4e82-a9ee-148d6cda19f1, null, Cell line from an NF1 patient; unclear if derived from tumor or non-tumor tissue., Neurofibromatosis type 1, null, f860ef0a-2501-4fa1-b0be-f638bde25c6d, null, null, null, null, null, null, null, [general NF1 deficiency], unknown, null, null, null, null, null, Asian, null, 1a8a362e-e3be-4bc3-be7e-d0a0865b9c31, NF1, cellLine, RRID:CVCL_JG80, Male, Homo sapiens, null, null, null, null, null, Not Applicable, [unknown], 247530ea-aba9-4560-9c67-39f6f9de532f, 111541ac-94a0-4190-babc-cf5be4bbd1ab, JCRB Cell Bank, https://cellbank.nibiohn.go.jp]
[Malignant soft-tissue tumors often present substantial diagnostic challenges. Chromosome aberrations that might be diagnostic have been identified in some types of soft-tissue tumors, but the overall frequency and diagnostic relevance of these aberrations have not been established. We attempted to determine the karyotypes of a series of 62 consecutive, unselected malignant spindle-cell or small round-cell soft-tissue tumors (from 46 adults and 16 children) after direct harvesting of cells or short-term culture. All tumors were examined independently by immunohistochemical staining in addition to routine light-microscopical evaluation, and all but two tumors were examined by electron microscopy. Metaphases were obtained from 61 of the 62 tumors, and clonal chromosome aberrations were identified in 55 (89 percent). In the six tumors that yielded metaphases but lacked apparent clonal aberrations, the normal metaphases were found to originate from non-neoplastic stromal elements within the tumor specimens. Thus, all tumors in which karyotyping was successful contained clonal chromosome aberrations. Forty of 62 tumors (65 percent) contained clonal chromosome aberrations that either suggested or confirmed a specific diagnosis; in 15 of these tumors (24 percent of all tumors), the aberrations were important in establishing the final diagnosis. Cytogenetic analyses were particularly informative about small round-cell tumors from children: 8 of 14 round-cell tumors contained diagnostically important chromosome aberrations. Using the combined approaches of light and electron microscopy, immunohistochemistry, and cytogenetics, we established an unambiguous diagnosis for 60 of 62 tumors. Cytogenetic analyses reveal clonal chromosome aberrations in virtually all malignant soft-tissue tumors. These clonal chromosome aberrations, particularly in small round-cell tumors in children, often have diagnostic relevance., null, null, [J A Fletcher, H P Kozakewich, F A Hoffer, J M Lage, N Weidner, R Tepper, G S Pinkus, C C Morton, J M Corson], null, null, Cancer cell line, ce2107ee-f903-4ed9-a6d7-3c91de703f4e, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1056/NEJM199102143240702, f926219f-4a17-45cc-a41a-aee6abb4c761, null, null, null, null, null, null, The New England Journal of Medicine, [malignant peripheral nerve sheath tumor], unknown, null, null, null, null, 0235bd13-fd94-4b95-9e98-19c1c4d9afc2, null, null, 202c110b-a5f1-49ab-acdc-e6e33a1c29bb, ST88-14, cellLine, RRID:CVCL_8916, Male, Homo sapiens, null, null, [ST88.14, ST 88-14, ST-8814, ST8814, 88-14, NF188-14], null, null, malignant peripheral nerve sheath tumor, [unknown], null, null, null, null]
[Malignant peripheral nerve sheath tumors (MPNST) are the most aggressive cancers associated with neurofibromatosis type 1 (NF1). Here we report a practical and reproducible model of intraneural NF1 MPNST, by orthotopic xenograft of an immortal human NF1 tumor-derived Schwann cell line into the sciatic nerves of female scid mice. Intraneural injection of the cell line sNF96.2 consistently produced MPNST-like tumors that were highly cellular and showed extensive intraneural growth. These xenografts had a high proliferative index, were angiogenic, had significant mast cell infiltration and rapidly dominated the host nerve. The histopathology of engrafted intraneural tumors was consistent with that of human NF1 MPNST. Xenograft tumors were readily examined by magnetic resonance imaging, which also was used to assess tumor vascularity. In addition, the intraneural proliferation of sNF96.2 cell tumors was decreased in ovariectomized mice, while replacement of estrogen or progesterone restored tumor cell proliferation. This suggests a potential role for steroid hormones in supporting tumor cell growth of this MPNST cell line in vivo. The controlled orthotopic implantation of sNF96.2 cells provides for the precise initiation of intraneural MPNST-like tumors in a model system suitable for therapeutic interventions, including inhibitors of angiogenesis and further study of steroid hormone effects on tumor cell growth., null, null, [George Q Perrin, Hua Li, Lauren Fishbein, Susanne A Thomson, Min S Hwang, Mark T Scarborough, Anthony T Yachnis, Margaret R Wallace, Thomas H Mareci, David Muir], CRL-2884, null, Cancer cell line, ca79ff41-46a9-4a9f-9a12-55810fd9267b, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1038/labinvest.3700675, 63858b37-331f-430d-bcfa-01f6836a6003, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [malignant peripheral nerve sheath tumor], yes, null, null, null, null, bdd70b67-9ef6-4809-ba5b-51bb39cf63a0, null, null, c9a87975-378b-4930-8fda-e5896c42c86c, sNF96.2, cellLine, RRID:CVCL_K281, Male, Homo sapiens, null, null, [SNF96.2, sNF96-2], null, null, malignant peripheral nerve sheath tumor, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 26dd6c4b-f37a-4376-bb29-c78ee6924034, ATCC, https://www.atcc.org/]
[The neurofibroma, a common feature of neurofibromatosis type 1 (NF1), is a benign peripheral nerve sheath tumor that contains predominantly Schwann cells (SC). There are reports that neurofibroma growth may be affected by hormonal changes, particularly in puberty and pregnancy, suggesting an influence by steroid hormones. This study examined the effects of estrogen and progesterone on proliferation and apoptosis in a panel of NF1 tumor xenografts. SC-enriched cultures derived from three human NF1 tumor types (dermal neurofibroma, plexiform neurofibroma, and malignant peripheral nerve sheath tumor (MPNST)) were xenografted in sciatic nerves of ovariectomized scid /Nf1-/+ mice. At the same time, mice were implanted with time-release pellets for systemic delivery of progesterone, estrogen or placebo. Proliferation and apoptosis by the xenografted SC were examined two months after implantation, by Ki67 immunolabeling and TUNEL. Estrogen was found to increase the growth of all three MPNST xenografts. Progesterone was associated with increased growth in two of the three MPNSTs, yet decreased growth of the other. Of the four dermal neurofibroma xenografts tested, estrogen caused a statistically significant growth increase in one, and progesterone did in another. Of the four plexiform neurofibroma SC xenografts, estrogen and progesterone significantly decreased growth in one of the xenografts, but not the other three. No relationship of patient age or gender to steroid response was observed. These findings indicate that human NF1 Schwann cells derived from some tumors show increased proliferation or decreased apoptosis in response to particular steroid hormones in a mouse xenograft model. This suggests that anti-estrogen or anti-progesterone therapies may be worth considering for specific NF1 neurofibromas and MPNSTs., null, null, [Hua Li, Xuelian Zhang, Lauren Fishbein, Frederick Kweh, Martha Campbell-Thompson, George Q Perrin, David Muir, Margaret Wallace], CRL-2885, null, Cancer cell line, 39b4b78a-44f4-4c19-8b62-89ca471fb5d1, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.4161/cbt.10.8.12878, 7cd65ff8-953a-40ed-b318-b8562c646b11, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Cancer Biology & Therapy, [malignant peripheral nerve sheath tumor], yes, null, null, null, null, c21f4170-a611-4062-a455-26e74560a4d4, null, null, b72d99fd-ee1e-42ec-92b6-9f89e375cce1, sNF02.2, cellLine, RRID:CVCL_K280, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, b603b0a5-cc1d-48b3-aa03-e70a7d19eb1e, ATCC, https://www.atcc.org/]

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[Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs., null, null, ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"], null, null, Cancer cell line, f16da853-b023-4c40-b632-4736222782d4, null, 827f08a1-3d0f-4fd8-98e4-fb5c40a9c742 RRID:CVCL_YU17 NCC-MPNST5-C1 null cellLine MPNST tumor cell line from an NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 87aa03a3-e7fb-4229-8b59-4b31a7dfd496 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Tadashi Kondo null https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/ 56811196-5155-4e8e-a8eb-6ad560c493c9 10.1186/s12935-020-1128-z ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"] Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs. Cancer Cell International f16da853-b023-4c40-b632-4736222782d4 null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1186/s12935-020-1128-z, 87aa03a3-e7fb-4229-8b59-4b31a7dfd496, null, null, 40, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Tadashi Kondo, https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/, Cancer Cell International, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, 56811196-5155-4e8e-a8eb-6ad560c493c9, null, null, 827f08a1-3d0f-4fd8-98e4-fb5c40a9c742, NCC-MPNST5-C1, cellLine, RRID:CVCL_YU17, Female, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]
[Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs., null, null, ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"], null, null, Cancer cell line, 727ef28f-4a4c-4f66-a0bd-19a247032255, null, 7fe31236-bc93-449b-b559-9394999be926 RRID:CVCL_YU14 NCC-MPNST3-C1 null cellLine MPNST tumor cell line from an NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 421820aa-3437-4f90-9bc8-f68c65d50a9a Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Tadashi Kondo null https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/ 56811196-5155-4e8e-a8eb-6ad560c493c9 10.1186/s12935-020-1128-z ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"] Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs. Cancer Cell International 727ef28f-4a4c-4f66-a0bd-19a247032255 null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1186/s12935-020-1128-z, 421820aa-3437-4f90-9bc8-f68c65d50a9a, null, null, 41, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Tadashi Kondo, https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/, Cancer Cell International, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, 56811196-5155-4e8e-a8eb-6ad560c493c9, null, null, 7fe31236-bc93-449b-b559-9394999be926, NCC-MPNST3-C1, cellLine, RRID:CVCL_YU14, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]
[Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs., null, null, ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"], null, null, Cancer cell line, 15be8727-dbbb-4ba1-a3ec-642b0a60100a, null, f17b839e-acd9-4dbc-a5bb-7d72ed0d0bd8 RRID:CVCL_YU16 NCC-MPNST4-C1 null cellLine MPNST tumor cell line from an NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 7da1348b-16ad-4d0d-9558-9f3395361518 Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Tadashi Kondo null https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/ 56811196-5155-4e8e-a8eb-6ad560c493c9 10.1186/s12935-020-1128-z ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"] Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs. Cancer Cell International 15be8727-dbbb-4ba1-a3ec-642b0a60100a null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1186/s12935-020-1128-z, 7da1348b-16ad-4d0d-9558-9f3395361518, null, null, 42, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Tadashi Kondo, https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/, Cancer Cell International, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, 56811196-5155-4e8e-a8eb-6ad560c493c9, null, null, f17b839e-acd9-4dbc-a5bb-7d72ed0d0bd8, NCC-MPNST4-C1, cellLine, RRID:CVCL_YU16, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]
[Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs., null, null, ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"], null, null, Cancer cell line, a6ca9a69-e262-45f6-bef9-836af7ab4cc4, null, 1b604846-d41c-4969-a79a-9660c04e585c RRID:CVCL_YU15 NCC-MPNST3-X2-C1 null cellLine MPNST tumor cell line from an NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 421820aa-3437-4f90-9bc8-f68c65d50a9a Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Tadashi Kondo null https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/ 56811196-5155-4e8e-a8eb-6ad560c493c9 10.1186/s12935-020-1128-z ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"] Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs. Cancer Cell International a6ca9a69-e262-45f6-bef9-836af7ab4cc4 null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1186/s12935-020-1128-z, 421820aa-3437-4f90-9bc8-f68c65d50a9a, null, null, 43, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Tadashi Kondo, https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/, Cancer Cell International, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, 56811196-5155-4e8e-a8eb-6ad560c493c9, null, null, 1b604846-d41c-4969-a79a-9660c04e585c, NCC-MPNST3-X2-C1, cellLine, RRID:CVCL_YU15, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]
[Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs., null, null, ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"], null, null, Cancer cell line, 177e3901-1fc6-4ad9-a088-babc8ff91a15, null, 8f646e44-5263-4f80-bb94-b297a875c52a RRID:CVCL_YU13 NCC-MPNST2-C1 null cellLine MPNST tumor cell line from an NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 b543357b-0345-4a8b-8ede-b0a8a30092b7 Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Tadashi Kondo null https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/ 56811196-5155-4e8e-a8eb-6ad560c493c9 10.1186/s12935-020-1128-z ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"] Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs. Cancer Cell International 177e3901-1fc6-4ad9-a088-babc8ff91a15 null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1186/s12935-020-1128-z, b543357b-0345-4a8b-8ede-b0a8a30092b7, null, null, 44, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Tadashi Kondo, https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/, Cancer Cell International, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, 56811196-5155-4e8e-a8eb-6ad560c493c9, null, null, 8f646e44-5263-4f80-bb94-b297a875c52a, NCC-MPNST2-C1, cellLine, RRID:CVCL_YU13, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]
[Skin fibroblast cell strains and tumour cell lines were established from 12 patients with various types of soft tissue neoplasms, and radiation survival curve parameters were measured in vitro. Soft tissue sarcoma cells were consistently more sensitive to X-irradiation than fibroblasts isolated from the same patient, and were also more sensitive as a group than cell lines derived from 34 other human tumours. There was a general correlation in radiosensitivity between fibroblasts and tumour cells derived from the same patient, indicating that some component of tumour cell sensitivity may relate to genetic factors in the host. Such genetic factors, however, do not explain all of the heterogeneity in tumour cell response. The response of soft tissue sarcoma in vivo may be dependent on complex radiomodifying factors other than inherent radiation sensitivity, thus making it difficult to predict clinical outcome by use of assays which use survival of irradiated tumour cell lines in vitro as an endpoint., null, null, ["W K Dahlberg", "J B Little", "J A Fletcher", "H D Suit", "P Okunieff"], null, null, Cancer cell line, 1139e45c-9645-449f-a22e-7497cbffb0b3, null, 9a7997c9-9399-47ed-b44b-5b717be89ba3 RRID:CVCL_8917 STS-26T ["STS26", "STS26T"] cellLine Sporadic MPNST tumor cell line from a non-NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Cancer b50e7512-a686-4c50-9e95-6af9d06bf826 Homo sapiens Female null null null null null null null null null null null null 979d13af-cd42-4fcf-ad7f-bd52e4b28bcf 10.1080/09553009314550251 ["W K Dahlberg", "J B Little", "J A Fletcher", "H D Suit", "P Okunieff"] Skin fibroblast cell strains and tumour cell lines were established from 12 patients with various types of soft tissue neoplasms, and radiation survival curve parameters were measured in vitro. Soft tissue sarcoma cells were consistently more sensitive to X-irradiation than fibroblasts isolated from the same patient, and were also more sensitive as a group than cell lines derived from 34 other human tumours. There was a general correlation in radiosensitivity between fibroblasts and tumour cells derived from the same patient, indicating that some component of tumour cell sensitivity may relate to genetic factors in the host. Such genetic factors, however, do not explain all of the heterogeneity in tumour cell response. The response of soft tissue sarcoma in vivo may be dependent on complex radiomodifying factors other than inherent radiation sensitivity, thus making it difficult to predict clinical outcome by use of assays which use survival of irradiated tumour cell lines in vitro as an endpoint. International Journal of Radiation Biology 1139e45c-9645-449f-a22e-7497cbffb0b3 null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , Sporadic MPNST tumor cell line from a non-NF1 patient., Cancer, 10.1080/09553009314550251, b50e7512-a686-4c50-9e95-6af9d06bf826, null, null, 45, null, null, null, null, International Journal of Radiation Biology, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, 979d13af-cd42-4fcf-ad7f-bd52e4b28bcf, null, null, 9a7997c9-9399-47ed-b44b-5b717be89ba3, STS-26T, cellLine, RRID:CVCL_8917, Female, Homo sapiens, null, null, ["STS26", "STS26T"], null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]
[Neurofibromatosis type 1 (NF1) is characterized by the formation of neurofibromas, benign tumors of the peripheral nerve consisting essentially of Schwann cells, which can sometimes turn malignant to form neurofibrosarcomas. The mechanism of progression toward a malignant phenotype remains largely unknown. In this report, we show that platelet-derived growth factor (PDGF) BB, and to a lesser extent fibroblast growth factor 2, are mitogenic for two neurofibrosarcoma-derived Schwann cell lines, but not for a Schwann cell line derived from a schwannoma (from a non-NF1 patient) or for transformed rat Schwann cells. Levels of expression of both PDGF receptor alpha and beta are significantly increased in the two neurofibrosarcoma-derived cell lines compared to the non-NF1 Schwann cell lines. The level of tyrosyl-phosphorylated PDGF receptor beta is strongly increased upon stimulation by PDGF BB. In comparison, only modest levels of tyrosyl-phosphorylated PDGF receptor alpha are observed, upon stimulation by PDGF AA or PDGF BB. Accordingly, PDGF AA is only a weak mitogen for the neurofibrosarcoma-derived cells by comparison to PDGF BB. These results indicate that the mitogenic effect of PDGF BB for the neurofibrosarcoma-derived Schwann cell lines is primarily transduced by PDGF receptor beta. Neu differentiation factor beta, a potent mitogen for normal Schwann cells, was unable to stimulate proliferation of the transformed Schwann cell lines, due to a dramatic down-regulation of the erbB3 receptor. Therefore, aberrant expression of growth factor receptors by Schwann cells, such as the PDGF receptors, could represent an important step in the process leading to Schwann cell hyperplasia in NF1., null, null, ["A Badache", "G H De Vries"], null, null, Cancer cell line, 394dd9ac-6a36-4515-bcc3-4012ba60c86d, null, 6419dd0d-1937-4ecf-bf01-876632ae0f54 RRID:CVCL_S805 T265 ["T265-2c", "T265-2C", "T265p21"] cellLine MPNST tumor cell line from an NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 18f6f681-54bc-431c-a447-3f3d4eae8229 Homo sapiens Unknown null null null null null null null null null null null null b9fae169-947b-413a-8d99-3dd094355943 10.1002/(SICI)1097-4652(199811)177:2<334::AID-JCP15>3.0.CO;2-9 ["A Badache", "G H De Vries"] Neurofibromatosis type 1 (NF1) is characterized by the formation of neurofibromas, benign tumors of the peripheral nerve consisting essentially of Schwann cells, which can sometimes turn malignant to form neurofibrosarcomas. The mechanism of progression toward a malignant phenotype remains largely unknown. In this report, we show that platelet-derived growth factor (PDGF) BB, and to a lesser extent fibroblast growth factor 2, are mitogenic for two neurofibrosarcoma-derived Schwann cell lines, but not for a Schwann cell line derived from a schwannoma (from a non-NF1 patient) or for transformed rat Schwann cells. Levels of expression of both PDGF receptor alpha and beta are significantly increased in the two neurofibrosarcoma-derived cell lines compared to the non-NF1 Schwann cell lines. The level of tyrosyl-phosphorylated PDGF receptor beta is strongly increased upon stimulation by PDGF BB. In comparison, only modest levels of tyrosyl-phosphorylated PDGF receptor alpha are observed, upon stimulation by PDGF AA or PDGF BB. Accordingly, PDGF AA is only a weak mitogen for the neurofibrosarcoma-derived cells by comparison to PDGF BB. These results indicate that the mitogenic effect of PDGF BB for the neurofibrosarcoma-derived Schwann cell lines is primarily transduced by PDGF receptor beta. Neu differentiation factor beta, a potent mitogen for normal Schwann cells, was unable to stimulate proliferation of the transformed Schwann cell lines, due to a dramatic down-regulation of the erbB3 receptor. Therefore, aberrant expression of growth factor receptors by Schwann cells, such as the PDGF receptors, could represent an important step in the process leading to Schwann cell hyperplasia in NF1. Journal of Cellular Physiology 394dd9ac-6a36-4515-bcc3-4012ba60c86d null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1002/(SICI)1097-4652(199811)177:2<334::AID-JCP15>3.0.CO;2-9, 18f6f681-54bc-431c-a447-3f3d4eae8229, null, null, 51, null, null, null, null, Journal of Cellular Physiology, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, b9fae169-947b-413a-8d99-3dd094355943, null, null, 6419dd0d-1937-4ecf-bf01-876632ae0f54, T265, cellLine, RRID:CVCL_S805, Unknown, Homo sapiens, null, null, ["T265-2c", "T265-2C", "T265p21"], null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d RRID:CVCL_UI69 hTERT NF1 ipnNF95.11c ["ipnNF95.11c", "ipnNF95.11C"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. yes ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null 9787b08c-6ecf-4159-8918-928c800ad977 CRL-3391 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology bacda034-66c2-4d1d-b235-7258682342a2 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 10, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, ["ipnNF95.11c", "ipnNF95.11C"], null, null, Not Applicable, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304 RRID:CVCL_UI73 hTERT NF1 ipnNF09.4 ["ipnNF09.4"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. unknown ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 55e157c9-c4c1-40d8-9187-6e6866c3ab46 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 12, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, ["ipnNF09.4"], null, null, Not Applicable, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, 98beda5b-9b28-4119-829a-2a0219d77af7 RRID:CVCL_UI75 hTERT NF1 sipnNF95.12B ["sipnNF95.12B"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. unknown ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4e75c936-aa0d-4689-a0fd-d41b0072bc5c Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 428d6875-ad27-4ed5-ac53-39cb73697ca6 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, 16, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, ["sipnNF95.12B"], null, null, Not Applicable, ["unknown"], null, null, null, null]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, [ipNF04.4], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 77bdc425-5f93-4fc0-9033-a3c63aa6da73, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 6c7d59b3-e542-40f3-9d5f-04ca85466b26, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, a8f48ce7-438c-4564-a70b-020abc96d5fe, hTERT NF1 ipNF00.6, cellLine, RRID:CVCL_UI76, Female, Homo sapiens, null, null, [ipNF00.6], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, a8e596b4-6067-4ac2-8d9c-7309f4b391ad, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c3fb3ba5-903b-4b31-a4f1-03462e18093f, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, f21cb7db-ee85-48a7-8f41-e4603238bede, hTERT NF1 ipNF03.3, cellLine, RRID:CVCL_UI77, Male, Homo sapiens, null, null, [ipNF03.3], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, [ipNF06.2A], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, [ipNF05.5 mixed clone, ipNF05.5 (mixed clone)], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, [ipNF05.5], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, [ipNF95.11b C/T, ipNF95.11bC_T], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, [ipNF95.11b C, ipNF95.11bC], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3389, null, Telomerase immortalized cell line, 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94, null, Derived from a plexiform neurofibroma growing on cranial nerve XII., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 108cfda9-d027-49cf-b950-d3e76097aa3c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, b44361f2-9021-4920-901a-b1a1f9143f97, hTERT NF1 ipNF95.6, cellLine, RRID:CVCL_UI70, Male, Homo sapiens, null, null, [ipNF95.6], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, e4426959-f253-4eac-8c68-3415f43104af, ATCC, https://www.atcc.org/]
[Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology. Introduction of a Synapsin I promoter driven Cre transgenic mouse strain into the conditional NF1 background has ablated NF1 function in most differentiated neuronal populations. These mice have abnormal development of the cerebral cortex, which suggests that NF1 has an indispensable role in this aspect of CNS development. Furthermore, although they are tumor free, these mice display extensive astrogliosis in the absence of conspicuous neurodegeneration or microgliosis. These results indicate that NF1-deficient neurons are capable of inducing reactive astrogliosis via a non-cell autonomous mechanism., b75c336e-791d-4a62-b556-153c88b28960, mouse cells, [Y Zhu, M I Romero, P Ghosh, Z Ye, P Charnay, E J Rushing, J D Marth, L F Parada], JAX_017640, https://www.jax.org/strain/017640, null, null, null, [From JAX]: These mice possess loxP sites flanking exons 31-32 of the neurofibromatosis 1 (Nf1) gene and have applications in studies of cancer, neural crest development and neurofibromatosis type I., Neurofibromatosis type 1, 10.1101/gad.862101, be180150-374d-4459-9a81-398c8a34bca9, null, C57BL/6J, UT Southwestern Medical Center, bb909638-4123-41a1-bd61-b300c13e7652, Luis Parada, null, Genes & Development, [plexiform neurofibroma], yes, null, null, null, null, b0332348-464f-4952-a7f7-2ffe4dc6b3f9, null, null, 118aa95e-27f4-48fa-94c7-5f786b8b4f2f, B6.129(Cg)-Nf1tm1Par/J, animalModel, RRID:IMSR_JAX:017640, null, Mus musculus, null, B6.129(Cg)-Nf1tm1Par/J, [Nf1flox], null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, f137e564-d395-4226-bc60-149c3c760e99, The Jackson Laboratory, https://www.jax.org]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, [ipNF04.4], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 77bdc425-5f93-4fc0-9033-a3c63aa6da73, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 6c7d59b3-e542-40f3-9d5f-04ca85466b26, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, a8f48ce7-438c-4564-a70b-020abc96d5fe, hTERT NF1 ipNF00.6, cellLine, RRID:CVCL_UI76, Female, Homo sapiens, null, null, [ipNF00.6], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, a8e596b4-6067-4ac2-8d9c-7309f4b391ad, null, Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c3fb3ba5-903b-4b31-a4f1-03462e18093f, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, f21cb7db-ee85-48a7-8f41-e4603238bede, hTERT NF1 ipNF03.3, cellLine, RRID:CVCL_UI77, Male, Homo sapiens, null, null, [ipNF03.3], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, [ipNF06.2A], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, [ipNF05.5 mixed clone, ipNF05.5 (mixed clone)], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, [ipNF05.5], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, [ipNF95.11b C/T, ipNF95.11bC_T], null, null, plexiform neurofibroma, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, [ipNF95.11b C, ipNF95.11bC], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]
[Plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1 (NF1) and have a risk of malignant progression. Past efforts to establish xenograft models for neurofibroma involved the implantation of tumor fragments or heterogeneous primary cultures, which rarely achieved significant tumor growth. We report a practical and reproducible animal model of plexiform-like neurofibroma by xenograft of an immortal human NF1 tumor-derived Schwann cell line into the peripheral nerve of scid mice. The S100 and p75 positive sNF94.3 cell line was shown to possess a normal karyotype and have apparent full-length neurofibromin by Western blot. These cells were shown to have a constitutional NF1 microdeletion and elevated Ras-GTP activity, however, suggesting loss of normal neurofibromin function. Localized intraneural injection of the cell line sNF94.3 produced consistent and slow growing tumors that infiltrated and disrupted the host nerve. The xenograft tumors resembled plexiform neurofibromas with a low rate of proliferation, abundant extracellular matrix (hypocellularity), basal laminae, high vascularity, and mast cell infiltration. The histologic features of the developed tumors were particularly consistent with those of human plexiform neurofibroma as well. Intraneural xenograft of sNF94.3 cells enables the precise initiation of intraneural, plexiform-like tumors and provides a highly reproducible model for the study of plexiform neurofibroma tumorigenesis. This model facilitates testing of potential therapeutic interventions, including angiogenesis inhibitors, in a relevant cellular environment., null, null, [George Q Perrin, Lauren Fishbein, Susanne A Thomson, Stacey L Thomas, Karen Stephens, James Y Garbern, George H DeVries, Anthony T Yachnis, Margaret R Wallace, David Muir], CRL-2886, null, Cancer cell line, 57f88a46-b615-41fa-9b89-9cdbc9dff0dc, null, MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1002/jnr.21226, 362b5ed5-90bd-47b6-89d7-0093f1b79204, null, null, null, https://www.atcc.org/, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Journal of Neuroscience Research, [malignant peripheral nerve sheath tumor], yes, null, null, null, null, 52529f5e-062b-40c9-9e87-ec52fe0ef918, null, null, 504647eb-6b60-492a-bb51-3ab025830f51, sNF94.3, cellLine, RRID:CVCL_K164, Female, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, e7389739-edb8-4e6c-9ad6-06bfb6b3db0e, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3389, null, Telomerase immortalized cell line, 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94, null, Derived from a plexiform neurofibroma growing on cranial nerve XII., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 108cfda9-d027-49cf-b950-d3e76097aa3c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [plexiform neurofibroma], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, b44361f2-9021-4920-901a-b1a1f9143f97, hTERT NF1 ipNF95.6, cellLine, RRID:CVCL_UI70, Male, Homo sapiens, null, null, [ipNF95.6], null, null, plexiform neurofibroma, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, e4426959-f253-4eac-8c68-3415f43104af, ATCC, https://www.atcc.org/]

[ABSTRACT, ANIMALMODELID, ANIMALSTATE, AUTHORS, CATALOGNUMBER, CATALOGNUMBERURL, CELLLINECATEGORY, CELLLINEID, CONTAMINATEDMISIDENTIFIED, CONTENT_TEXT, DESCRIPTION, DISEASE, DOI, DONORID, GENERATION, GENETICBACKGROUND, ID, INSTITUTION, INVESTIGATORID, INVESTIGATORNAME, INVESTIGATORWEBSITE, JOURNAL, MODELOFMANIFESTATION, MTAREQUIRED, ORCID, ORGAN, ORIGINYEAR, POPULATIONDOUBLINGTIME, PUBLICATIONID, RACE, RESISTANCE, RESOURCEID, RESOURCENAME, RESOURCETYPE, RRID, SEX, SPECIES, STRAINNOMENCLATURE, STRPROFILE, SYNONYMS, TISSUE, TRANSPLANTATIONTYPE, TUMORTYPE, USAGERESTRICTIONS, VENDORID, VENDORITEMID, VENDORNAME, WEBSITE]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3387, null, Telomerase immortalized cell line, 71e33e5d-2392-4112-b284-1386842dd935, null, 844b598c-0171-4972-91c3-27aa21b45d52 RRID:CVCL_UI72 hTERT NF1 ipNF05.5 mixed clones ["ipNF05.5 mixed clone", "ipNF05.5 (mixed clone)"] cellLine Derived from a plexiform neurofibroma growing on a hand. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null 9a08aa2d-3d44-497f-9d49-12e9b30d82cf CRL-3387 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 71e33e5d-2392-4112-b284-1386842dd935 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 7, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 844b598c-0171-4972-91c3-27aa21b45d52, hTERT NF1 ipNF05.5 mixed clones, cellLine, RRID:CVCL_UI72, Male, Homo sapiens, null, null, ["ipNF05.5 mixed clone", "ipNF05.5 (mixed clone)"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 9a08aa2d-3d44-497f-9d49-12e9b30d82cf, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, c5037bab-6cce-49f6-91ab-2ac26753829b, null, ab60aae5-7860-4d1d-bb02-208e6631c78b RRID:CVCL_UI68 hTERT NF1 ipNF95.11b C/T ["ipNF95.11b C/T", "ipNF95.11bC_T"] cellLine Derived from a plexiform neurofibroma growing on a brachial plexus. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology c5037bab-6cce-49f6-91ab-2ac26753829b null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 8, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, ab60aae5-7860-4d1d-bb02-208e6631c78b, hTERT NF1 ipNF95.11b C/T, cellLine, RRID:CVCL_UI68, Male, Homo sapiens, null, null, ["ipNF95.11b C/T", "ipNF95.11bC_T"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3390, null, Telomerase immortalized cell line, 36bd1db8-c1af-4825-a557-13d6276f7949, null, be2333d6-6716-4d13-947d-41f4198497a4 RRID:CVCL_UI67 hTERT NF1 ipNF95.11b C ["ipNF95.11b C", "ipNF95.11bC"] cellLine Derived from a plexiform neurofibroma growing on a brachial plexus. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null f67f594e-b2c0-4497-a5d9-4699b3b2d601 CRL-3390 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 36bd1db8-c1af-4825-a557-13d6276f7949 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a brachial plexus., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 9, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, be2333d6-6716-4d13-947d-41f4198497a4, hTERT NF1 ipNF95.11b C, cellLine, RRID:CVCL_UI67, Male, Homo sapiens, null, null, ["ipNF95.11b C", "ipNF95.11bC"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, f67f594e-b2c0-4497-a5d9-4699b3b2d601, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3388, null, Telomerase immortalized cell line, d43864f1-277e-41dd-ae77-2d8b153b9be8, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f RRID:CVCL_UI71 hTERT NF1 ipNF05.5 ["ipNF05.5"] cellLine Derived from a plexiform neurofibroma growing on a hand. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null 071ef5b1-79cb-4a4d-ad61-900eea8f9be0 CRL-3388 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology d43864f1-277e-41dd-ae77-2d8b153b9be8 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on a hand., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 11, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 5502caf5-4cf1-418f-bf50-164cfa316b0f, hTERT NF1 ipNF05.5, cellLine, RRID:CVCL_UI71, Male, Homo sapiens, null, null, ["ipNF05.5"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 071ef5b1-79cb-4a4d-ad61-900eea8f9be0, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 4d77247b-d123-42cf-8110-d335d0a9c953, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d RRID:CVCL_UI78 hTERT NF1 ipNF04.4 ["ipNF04.4"] cellLine Derived from a plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 c6a7d35b-580c-41ca-bf82-b5bfd5234289 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 4d77247b-d123-42cf-8110-d335d0a9c953 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c6a7d35b-580c-41ca-bf82-b5bfd5234289, null, null, 13, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8fdc5f7a-ef8c-4193-a5c0-04577c3b134d, hTERT NF1 ipNF04.4, cellLine, RRID:CVCL_UI78, Female, Homo sapiens, null, null, ["ipNF04.4"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3389, null, Telomerase immortalized cell line, 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94, null, b44361f2-9021-4920-901a-b1a1f9143f97 RRID:CVCL_UI70 hTERT NF1 ipNF95.6 ["ipNF95.6"] cellLine Derived from a plexiform neurofibroma growing on cranial nerve XII. yes ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 108cfda9-d027-49cf-b950-d3e76097aa3c Homo sapiens Male null e4426959-f253-4eac-8c68-3415f43104af CRL-3389 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 7cc21ef8-721e-4f62-a6a1-dfc7c6dd3f94 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma growing on cranial nerve XII., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 108cfda9-d027-49cf-b950-d3e76097aa3c, null, null, 14, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, b44361f2-9021-4920-901a-b1a1f9143f97, hTERT NF1 ipNF95.6, cellLine, RRID:CVCL_UI70, Male, Homo sapiens, null, null, ["ipNF95.6"], null, null, plexiform neurofibroma, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, e4426959-f253-4eac-8c68-3415f43104af, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, faff6884-47dd-4bbf-ab57-f420d87d456e, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb RRID:CVCL_UI74 hTERT NF1 ipNF06.2A ["ipNF06.2A"] cellLine Derived from a pleural plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 10a5e6f2-d331-4b5e-9e43-888cca69d5a9 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology faff6884-47dd-4bbf-ab57-f420d87d456e null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a pleural plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 10a5e6f2-d331-4b5e-9e43-888cca69d5a9, null, null, 15, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 3dedf9d2-614c-4cf9-8d18-aff8aa2dc0eb, hTERT NF1 ipNF06.2A, cellLine, RRID:CVCL_UI74, Female, Homo sapiens, null, null, ["ipNF06.2A"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 77bdc425-5f93-4fc0-9033-a3c63aa6da73, null, a8f48ce7-438c-4564-a70b-020abc96d5fe RRID:CVCL_UI76 hTERT NF1 ipNF00.6 ["ipNF00.6"] cellLine Derived from a plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 6c7d59b3-e542-40f3-9d5f-04ca85466b26 Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 77bdc425-5f93-4fc0-9033-a3c63aa6da73 null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 6c7d59b3-e542-40f3-9d5f-04ca85466b26, null, null, 17, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, a8f48ce7-438c-4564-a70b-020abc96d5fe, hTERT NF1 ipNF00.6, cellLine, RRID:CVCL_UI76, Female, Homo sapiens, null, null, ["ipNF00.6"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, a8e596b4-6067-4ac2-8d9c-7309f4b391ad, null, f21cb7db-ee85-48a7-8f41-e4603238bede RRID:CVCL_UI77 hTERT NF1 ipNF03.3 ["ipNF03.3"] cellLine Derived from a plexiform neurofibroma. unknown ["unknown"] null null null null null null ["plexiform neurofibroma"] Neurofibromatosis type 1 c3fb3ba5-903b-4b31-a4f1-03462e18093f Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology a8e596b4-6067-4ac2-8d9c-7309f4b391ad null null Telomerase immortalized cell line null null null null null plexiform neurofibroma , Derived from a plexiform neurofibroma., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, c3fb3ba5-903b-4b31-a4f1-03462e18093f, null, null, 18, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["plexiform neurofibroma"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, f21cb7db-ee85-48a7-8f41-e4603238bede, hTERT NF1 ipNF03.3, cellLine, RRID:CVCL_UI77, Male, Homo sapiens, null, null, ["ipNF03.3"], null, null, plexiform neurofibroma, ["unknown"], null, null, null, null]
[Plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1 (NF1) and have a risk of malignant progression. Past efforts to establish xenograft models for neurofibroma involved the implantation of tumor fragments or heterogeneous primary cultures, which rarely achieved significant tumor growth. We report a practical and reproducible animal model of plexiform-like neurofibroma by xenograft of an immortal human NF1 tumor-derived Schwann cell line into the peripheral nerve of scid mice. The S100 and p75 positive sNF94.3 cell line was shown to possess a normal karyotype and have apparent full-length neurofibromin by Western blot. These cells were shown to have a constitutional NF1 microdeletion and elevated Ras-GTP activity, however, suggesting loss of normal neurofibromin function. Localized intraneural injection of the cell line sNF94.3 produced consistent and slow growing tumors that infiltrated and disrupted the host nerve. The xenograft tumors resembled plexiform neurofibromas with a low rate of proliferation, abundant extracellular matrix (hypocellularity), basal laminae, high vascularity, and mast cell infiltration. The histologic features of the developed tumors were particularly consistent with those of human plexiform neurofibroma as well. Intraneural xenograft of sNF94.3 cells enables the precise initiation of intraneural, plexiform-like tumors and provides a highly reproducible model for the study of plexiform neurofibroma tumorigenesis. This model facilitates testing of potential therapeutic interventions, including angiogenesis inhibitors, in a relevant cellular environment., null, null, ["George Q Perrin", "Lauren Fishbein", "Susanne A Thomson", "Stacey L Thomas", "Karen Stephens", "James Y Garbern", "George H DeVries", "Anthony T Yachnis", "Margaret R Wallace", "David Muir"], CRL-2886, null, Cancer cell line, 57f88a46-b615-41fa-9b89-9cdbc9dff0dc, null, 504647eb-6b60-492a-bb51-3ab025830f51 RRID:CVCL_K164 sNF94.3 null cellLine MPNST tumor cell line from an NF1 patient. yes ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 362b5ed5-90bd-47b6-89d7-0093f1b79204 Homo sapiens Female null e7389739-edb8-4e6c-9ad6-06bfb6b3db0e CRL-2886 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ https://www.atcc.org/ null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 52529f5e-062b-40c9-9e87-ec52fe0ef918 10.1002/jnr.21226 ["George Q Perrin", "Lauren Fishbein", "Susanne A Thomson", "Stacey L Thomas", "Karen Stephens", "James Y Garbern", "George H DeVries", "Anthony T Yachnis", "Margaret R Wallace", "David Muir"] Plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1 (NF1) and have a risk of malignant progression. Past efforts to establish xenograft models for neurofibroma involved the implantation of tumor fragments or heterogeneous primary cultures, which rarely achieved significant tumor growth. We report a practical and reproducible animal model of plexiform-like neurofibroma by xenograft of an immortal human NF1 tumor-derived Schwann cell line into the peripheral nerve of scid mice. The S100 and p75 positive sNF94.3 cell line was shown to possess a normal karyotype and have apparent full-length neurofibromin by Western blot. These cells were shown to have a constitutional NF1 microdeletion and elevated Ras-GTP activity, however, suggesting loss of normal neurofibromin function. Localized intraneural injection of the cell line sNF94.3 produced consistent and slow growing tumors that infiltrated and disrupted the host nerve. The xenograft tumors resembled plexiform neurofibromas with a low rate of proliferation, abundant extracellular matrix (hypocellularity), basal laminae, high vascularity, and mast cell infiltration. The histologic features of the developed tumors were particularly consistent with those of human plexiform neurofibroma as well. Intraneural xenograft of sNF94.3 cells enables the precise initiation of intraneural, plexiform-like tumors and provides a highly reproducible model for the study of plexiform neurofibroma tumorigenesis. This model facilitates testing of potential therapeutic interventions, including angiogenesis inhibitors, in a relevant cellular environment. Journal of Neuroscience Research 57f88a46-b615-41fa-9b89-9cdbc9dff0dc null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1002/jnr.21226, 362b5ed5-90bd-47b6-89d7-0093f1b79204, null, null, 29, null, https://www.atcc.org/, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Journal of Neuroscience Research, ["malignant peripheral nerve sheath tumor"], yes, null, null, null, null, 52529f5e-062b-40c9-9e87-ec52fe0ef918, null, null, 504647eb-6b60-492a-bb51-3ab025830f51, sNF94.3, cellLine, RRID:CVCL_K164, Female, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, e7389739-edb8-4e6c-9ad6-06bfb6b3db0e, ATCC, https://www.atcc.org/]

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[Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs., null, null, ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"], null, null, Cancer cell line, a6ca9a69-e262-45f6-bef9-836af7ab4cc4, null, 1b604846-d41c-4969-a79a-9660c04e585c RRID:CVCL_YU15 NCC-MPNST3-X2-C1 null cellLine MPNST tumor cell line from an NF1 patient. unknown ["unknown"] null null null null null null ["malignant peripheral nerve sheath tumor"] Neurofibromatosis type 1 421820aa-3437-4f90-9bc8-f68c65d50a9a Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Tadashi Kondo null https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/ 56811196-5155-4e8e-a8eb-6ad560c493c9 10.1186/s12935-020-1128-z ["Rieko Oyama", "Fusako Kito", "Mami Takahashi", "Emi Hattori", "Rei Noguchi", "Yoko Takai", "Marimu Sakumoto", "Zhiwei Qiao", "Shunichi Toki", "Masato Sugawara", "Yoshikazu Tanzawa", "Eisuke Kobayashi", "Fumihiko Nakatani", "Shintaro Iwata", "Akihiko Yoshida", "Akira Kawai", "Tadashi Kondo"] Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs. Cancer Cell International a6ca9a69-e262-45f6-bef9-836af7ab4cc4 null null Cancer cell line null null null null null malignant peripheral nerve sheath tumor , MPNST tumor cell line from an NF1 patient., Neurofibromatosis type 1, 10.1186/s12935-020-1128-z, 421820aa-3437-4f90-9bc8-f68c65d50a9a, null, null, 43, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Tadashi Kondo, https://www.ncc.go.jp/jp/ri/division/rare_cancer_research/, Cancer Cell International, ["malignant peripheral nerve sheath tumor"], unknown, null, null, null, null, 56811196-5155-4e8e-a8eb-6ad560c493c9, null, null, 1b604846-d41c-4969-a79a-9660c04e585c, NCC-MPNST3-X2-C1, cellLine, RRID:CVCL_YU15, Male, Homo sapiens, null, null, null, null, null, malignant peripheral nerve sheath tumor, ["unknown"], null, null, null, null]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, [ipnNF95.11c, ipnNF95.11C], null, null, Not Applicable, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, [ipnNF09.4], null, null, Not Applicable, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, [sipnNF95.12B], null, null, Not Applicable, [unknown], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 9fc0cb6e-c5f6-4f08-a9d0-8f5e046659ab, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 4edead03-da70-41fa-829d-2bd511e9f3c1, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, 33cdb482-618e-4489-87d9-3139cc7c6a49, SZ-NF1, cellLine, RRID:CVCL_YL57, Unknown, Homo sapiens, null, null, [SZ-NF1 PGD, NF#1], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 3a7053c7-05f4-4a29-9957-587d51240a92, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 5b3c4bf5-21a8-4020-8e6f-bed3be6dd7c6, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, cc2045df-5ebf-4491-b5b1-88d65e59b228, SZ-NF2, cellLine, RRID:CVCL_YL58, Unknown, Homo sapiens, null, null, [SZ-NF2 PGD, NF#2], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 0ded24be-ba48-484f-95b5-4dcb995e708a, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 1420b3e6-04f9-4e8c-846b-04968327e31f, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, 09c988ab-765a-44ca-b2d7-1957b729208e, SZ-NF4, cellLine, RRID:CVCL_YL59, Unknown, Homo sapiens, null, null, [SZ-NF4 PGD, NF#4], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, JCRB3015, null, Finite cell line, 50ca52b3-8448-4e82-a9ee-148d6cda19f1, null, Cell line from an NF1 patient; unclear if derived from tumor or non-tumor tissue., Neurofibromatosis type 1, null, f860ef0a-2501-4fa1-b0be-f638bde25c6d, null, null, null, null, null, null, null, [general NF1 deficiency], unknown, null, null, null, null, null, Asian, null, 1a8a362e-e3be-4bc3-be7e-d0a0865b9c31, NF1, cellLine, RRID:CVCL_JG80, Male, Homo sapiens, null, null, null, null, null, Not Applicable, [unknown], 247530ea-aba9-4560-9c67-39f6f9de532f, 111541ac-94a0-4190-babc-cf5be4bbd1ab, JCRB Cell Bank, https://cellbank.nibiohn.go.jp]
[null, null, null, null, null, null, Embryonic stem cell, 7d6c779a-053e-448d-9a61-203d6c72decc, null, Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b5b23dce-2d21-4490-b9eb-95c46e2ac0eb, null, null, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, cd38ffda-2db1-47f0-af6f-8de572e06037, Lis42_NF1_1N, cellLine, RRID:CVCL_Y368, Unknown, Homo sapiens, null, null, [Lis42_NF1_1_N, Lis 42_NF1_1_N], null, null, Not Applicable, [none], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 4bd71d55-44c3-4a17-b921-5510932b9d1e, null, Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b6f8726c-3d3a-43ec-a2c3-46d4c56cf06e, null, null, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, d0ca95f1-a3d9-4641-b47d-84346d9ec04a, Lis47_NF1_2N, cellLine, RRID:CVCL_Y373, Unknown, Homo sapiens, null, null, [Lis47_NF1_2_N, Lis 47_NF1_1_N], null, null, Not Applicable, [none], null, null, null, null]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, [Heema Hewitson, Victoria Wood, Neli Kadeva, Glenda Cornwell, Stefano Codognotto, Emma Stephenson, Dusko Ilic], null, null, Embryonic stem cell, 86d1445f-0572-44c0-9301-75da0c1856fd, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 4442c058-074b-4b2e-9bb3-80a1a6cb75d1, null, null, null, null, null, null, Stem Cell Research, [general NF1 deficiency], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, e5fdcfb8-24e7-46fa-9f48-bcbae8a90b7a, KCL024, cellLine, RRID:CVCL_A257, Unknown, Homo sapiens, null, null, [KCL-024, KCL024_NF1-1], null, null, Not Applicable, [cells cannot be used for treatment purposes], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology. Introduction of a Synapsin I promoter driven Cre transgenic mouse strain into the conditional NF1 background has ablated NF1 function in most differentiated neuronal populations. These mice have abnormal development of the cerebral cortex, which suggests that NF1 has an indispensable role in this aspect of CNS development. Furthermore, although they are tumor free, these mice display extensive astrogliosis in the absence of conspicuous neurodegeneration or microgliosis. These results indicate that NF1-deficient neurons are capable of inducing reactive astrogliosis via a non-cell autonomous mechanism., b75c336e-791d-4a62-b556-153c88b28960, mouse cells, [Y Zhu, M I Romero, P Ghosh, Z Ye, P Charnay, E J Rushing, J D Marth, L F Parada], JAX_017640, https://www.jax.org/strain/017640, null, null, null, [From JAX]: These mice possess loxP sites flanking exons 31-32 of the neurofibromatosis 1 (Nf1) gene and have applications in studies of cancer, neural crest development and neurofibromatosis type I., Neurofibromatosis type 1, 10.1101/gad.862101, be180150-374d-4459-9a81-398c8a34bca9, null, C57BL/6J, UT Southwestern Medical Center, bb909638-4123-41a1-bd61-b300c13e7652, Luis Parada, null, Genes & Development, [plexiform neurofibroma], yes, null, null, null, null, b0332348-464f-4952-a7f7-2ffe4dc6b3f9, null, null, 118aa95e-27f4-48fa-94c7-5f786b8b4f2f, B6.129(Cg)-Nf1tm1Par/J, animalModel, RRID:IMSR_JAX:017640, null, Mus musculus, null, B6.129(Cg)-Nf1tm1Par/J, [Nf1flox], null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, f137e564-d395-4226-bc60-149c3c760e99, The Jackson Laboratory, https://www.jax.org]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, [ipnNF95.11c, ipnNF95.11C], null, null, Not Applicable, [unknown], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, [ipnNF09.4], null, null, Not Applicable, [unknown], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, [Hua Li, Lung-Ji Chang, Debbie R Neubauer, David F Muir, Margaret R Wallace], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, [general NF1 deficiency], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, [sipnNF95.12B], null, null, Not Applicable, [unknown], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 9fc0cb6e-c5f6-4f08-a9d0-8f5e046659ab, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 4edead03-da70-41fa-829d-2bd511e9f3c1, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, 33cdb482-618e-4489-87d9-3139cc7c6a49, SZ-NF1, cellLine, RRID:CVCL_YL57, Unknown, Homo sapiens, null, null, [SZ-NF1 PGD, NF#1], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 3a7053c7-05f4-4a29-9957-587d51240a92, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 5b3c4bf5-21a8-4020-8e6f-bed3be6dd7c6, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, cc2045df-5ebf-4491-b5b1-88d65e59b228, SZ-NF2, cellLine, RRID:CVCL_YL58, Unknown, Homo sapiens, null, null, [SZ-NF2 PGD, NF#2], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 0ded24be-ba48-484f-95b5-4dcb995e708a, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 1420b3e6-04f9-4e8c-846b-04968327e31f, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, 09c988ab-765a-44ca-b2d7-1957b729208e, SZ-NF4, cellLine, RRID:CVCL_YL59, Unknown, Homo sapiens, null, null, [SZ-NF4 PGD, NF#4], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, JCRB3015, null, Finite cell line, 50ca52b3-8448-4e82-a9ee-148d6cda19f1, null, Cell line from an NF1 patient; unclear if derived from tumor or non-tumor tissue., Neurofibromatosis type 1, null, f860ef0a-2501-4fa1-b0be-f638bde25c6d, null, null, null, null, null, null, null, [general NF1 deficiency], unknown, null, null, null, null, null, Asian, null, 1a8a362e-e3be-4bc3-be7e-d0a0865b9c31, NF1, cellLine, RRID:CVCL_JG80, Male, Homo sapiens, null, null, null, null, null, Not Applicable, [unknown], 247530ea-aba9-4560-9c67-39f6f9de532f, 111541ac-94a0-4190-babc-cf5be4bbd1ab, JCRB Cell Bank, https://cellbank.nibiohn.go.jp]
[null, null, null, null, null, null, Embryonic stem cell, 7d6c779a-053e-448d-9a61-203d6c72decc, null, Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b5b23dce-2d21-4490-b9eb-95c46e2ac0eb, null, null, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, cd38ffda-2db1-47f0-af6f-8de572e06037, Lis42_NF1_1N, cellLine, RRID:CVCL_Y368, Unknown, Homo sapiens, null, null, [Lis42_NF1_1_N, Lis 42_NF1_1_N], null, null, Not Applicable, [none], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 4bd71d55-44c3-4a17-b921-5510932b9d1e, null, Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b6f8726c-3d3a-43ec-a2c3-46d4c56cf06e, null, null, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, d0ca95f1-a3d9-4641-b47d-84346d9ec04a, Lis47_NF1_2N, cellLine, RRID:CVCL_Y373, Unknown, Homo sapiens, null, null, [Lis47_NF1_2_N, Lis 47_NF1_1_N], null, null, Not Applicable, [none], null, null, null, null]

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[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], CRL-3391, null, Telomerase immortalized cell line, bacda034-66c2-4d1d-b235-7258682342a2, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d RRID:CVCL_UI69 hTERT NF1 ipnNF95.11c ["ipnNF95.11c", "ipnNF95.11C"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. yes ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4fdcbad1-477b-46ce-82e6-7d0550383b46 Homo sapiens Male null 9787b08c-6ecf-4159-8918-928c800ad977 CRL-3391 null 17f67735-795a-4f94-971b-bc1e8c290674 ATCC https://www.atcc.org/ 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology bacda034-66c2-4d1d-b235-7258682342a2 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4fdcbad1-477b-46ce-82e6-7d0550383b46, null, null, 10, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], yes, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 2c4c8b02-f12e-4a17-a408-38ae88dd841d, hTERT NF1 ipnNF95.11c, cellLine, RRID:CVCL_UI69, Male, Homo sapiens, null, null, ["ipnNF95.11c", "ipnNF95.11C"], null, null, Not Applicable, ["unknown"], 17f67735-795a-4f94-971b-bc1e8c290674, 9787b08c-6ecf-4159-8918-928c800ad977, ATCC, https://www.atcc.org/]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 55e157c9-c4c1-40d8-9187-6e6866c3ab46, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304 RRID:CVCL_UI73 hTERT NF1 ipnNF09.4 ["ipnNF09.4"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. unknown ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 45f0b93c-a50b-45b0-b235-cc195d76aa48 Homo sapiens Male null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 55e157c9-c4c1-40d8-9187-6e6866c3ab46 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 45f0b93c-a50b-45b0-b235-cc195d76aa48, null, null, 12, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 8e0602ff-e3e6-438e-9fb7-c7abc1dd4304, hTERT NF1 ipnNF09.4, cellLine, RRID:CVCL_UI73, Male, Homo sapiens, null, null, ["ipnNF09.4"], null, null, Not Applicable, ["unknown"], null, null, null, null]
[Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions., null, null, ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"], null, null, Telomerase immortalized cell line, 428d6875-ad27-4ed5-ac53-39cb73697ca6, null, 98beda5b-9b28-4119-829a-2a0219d77af7 RRID:CVCL_UI75 hTERT NF1 sipnNF95.12B ["sipnNF95.12B"] cellLine Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation. unknown ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4e75c936-aa0d-4689-a0fd-d41b0072bc5c Homo sapiens Female null null null null null null null 2a6c6b46-453b-48a8-84c1-0468c3a83e7b null Margaret Wallace null http://mgm.ufl.edu/faculty/wallace-margaret-peggy/ 3df2ebce-4581-4f5c-84ae-45d31bbc97bb 10.1038/labinvest.2016.88 ["Hua Li", "Lung-Ji Chang", "Debbie R Neubauer", "David F Muir", "Margaret R Wallace"] Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic loss of NF1 gene function. There is a need for effective therapies for neurofibromas, particularly the larger, plexiform neurofibromas. Tissue culture is an important tool for research. However, it is difficult to derive enriched human Schwann cell cultures, and most enter replicative senescence after 6-10 passages, impeding cell-based research in NF1. Through exogenous expression of human telomerase reverse transcriptase and murine cyclin-dependent kinase (mCdk4), normal (NF1 wild-type), neurofibroma-derived Schwann cells heterozygous for NF1 mutation, and neurofibroma-derived Schwann cells homozygous for NF1 mutation were immortalized, including some matched samples from the same NF1 patient. Initial experiments employed retroviral vectors, while subsequent work utilized lentiviral vectors carrying these genes because of improved efficiency. Expression of both transgenes was required for immortalization. Molecular and immunohistochemical analysis indicated that these cell lines are of Schwann cell lineage and have a range of phenotypes, many of which are consistent with their primary cultures. This is the first report of immortalization and detailed characterization of multiple human NF1 normal nerve and neurofibroma-derived Schwann cell lines, which will be highly useful research tools to study NF1 and other Schwann tumor biology and conditions. Laboratory Investigation; a Journal of Technical Methods and Pathology 428d6875-ad27-4ed5-ac53-39cb73697ca6 null null Telomerase immortalized cell line null null null null null Not Applicable , Derived from a peripheral nerve in a neurofibromatosis type 1 patient. No detectable somatic NF1 mutation., Neurofibromatosis type 1, 10.1038/labinvest.2016.88, 4e75c936-aa0d-4689-a0fd-d41b0072bc5c, null, null, 16, null, 2a6c6b46-453b-48a8-84c1-0468c3a83e7b, Margaret Wallace, http://mgm.ufl.edu/faculty/wallace-margaret-peggy/, Laboratory Investigation; a Journal of Technical Methods and Pathology, ["general NF1 deficiency"], unknown, null, null, null, null, 3df2ebce-4581-4f5c-84ae-45d31bbc97bb, null, null, 98beda5b-9b28-4119-829a-2a0219d77af7, hTERT NF1 sipnNF95.12B, cellLine, RRID:CVCL_UI75, Female, Homo sapiens, null, null, ["sipnNF95.12B"], null, null, Not Applicable, ["unknown"], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 9fc0cb6e-c5f6-4f08-a9d0-8f5e046659ab, null, 33cdb482-618e-4489-87d9-3139cc7c6a49 RRID:CVCL_YL57 SZ-NF1 ["SZ-NF1 PGD", "NF#1"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. yes ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4edead03-da70-41fa-829d-2bd511e9f3c1 Homo sapiens Unknown null null null null null null null 247530ea-aba9-4560-9c67-39f6f9de532f null Rachel Eiges null https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/ null null null null null 9fc0cb6e-c5f6-4f08-a9d0-8f5e046659ab null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 4edead03-da70-41fa-829d-2bd511e9f3c1, null, null, 20, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, 33cdb482-618e-4489-87d9-3139cc7c6a49, SZ-NF1, cellLine, RRID:CVCL_YL57, Unknown, Homo sapiens, null, null, ["SZ-NF1 PGD", "NF#1"], null, null, Not Applicable, ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"], null, null, null, null]
[null, null, null, null, JCRB3015, null, Finite cell line, 50ca52b3-8448-4e82-a9ee-148d6cda19f1, null, 1a8a362e-e3be-4bc3-be7e-d0a0865b9c31 RRID:CVCL_JG80 NF1 null cellLine Cell line from an NF1 patient; unclear if derived from tumor or non-tumor tissue. unknown ["unknown"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 f860ef0a-2501-4fa1-b0be-f638bde25c6d Homo sapiens Male Asian 111541ac-94a0-4190-babc-cf5be4bbd1ab JCRB3015 null 247530ea-aba9-4560-9c67-39f6f9de532f JCRB Cell Bank https://cellbank.nibiohn.go.jp null null null null null null null null null null 50ca52b3-8448-4e82-a9ee-148d6cda19f1 null null Finite cell line null null null null null Not Applicable , Cell line from an NF1 patient; unclear if derived from tumor or non-tumor tissue., Neurofibromatosis type 1, null, f860ef0a-2501-4fa1-b0be-f638bde25c6d, null, null, 23, null, null, null, null, null, ["general NF1 deficiency"], unknown, null, null, null, null, null, Asian, null, 1a8a362e-e3be-4bc3-be7e-d0a0865b9c31, NF1, cellLine, RRID:CVCL_JG80, Male, Homo sapiens, null, null, null, null, null, Not Applicable, ["unknown"], 247530ea-aba9-4560-9c67-39f6f9de532f, 111541ac-94a0-4190-babc-cf5be4bbd1ab, JCRB Cell Bank, https://cellbank.nibiohn.go.jp]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, ["Heema Hewitson", "Victoria Wood", "Neli Kadeva", "Glenda Cornwell", "Stefano Codognotto", "Emma Stephenson", "Dusko Ilic"], null, null, Embryonic stem cell, 86d1445f-0572-44c0-9301-75da0c1856fd, null, e5fdcfb8-24e7-46fa-9f48-bcbae8a90b7a RRID:CVCL_A257 KCL024 ["KCL-024", "KCL024_NF1-1"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. no ["cells cannot be used for treatment purposes"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4442c058-074b-4b2e-9bb3-80a1a6cb75d1 Homo sapiens Unknown null null null null 0bf052d2-213f-46fd-8598-ff39c9aacaa9 King's College London https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx null null null null null 8c670bac-17e2-4d75-bc5c-9e5788f293b0 10.1016/j.scr.2016.01.010 ["Heema Hewitson", "Victoria Wood", "Neli Kadeva", "Glenda Cornwell", "Stefano Codognotto", "Emma Stephenson", "Dusko Ilic"] The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays. Stem Cell Research 86d1445f-0572-44c0-9301-75da0c1856fd null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 4442c058-074b-4b2e-9bb3-80a1a6cb75d1, null, null, 32, null, null, null, null, Stem Cell Research, ["general NF1 deficiency"], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, e5fdcfb8-24e7-46fa-9f48-bcbae8a90b7a, KCL024, cellLine, RRID:CVCL_A257, Unknown, Homo sapiens, null, null, ["KCL-024", "KCL024_NF1-1"], null, null, Not Applicable, ["cells cannot be used for treatment purposes"], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, ["Heema Hewitson", "Victoria Wood", "Neli Kadeva", "Glenda Cornwell", "Stefano Codognotto", "Emma Stephenson", "Dusko Ilic"], null, null, Embryonic stem cell, efcb4b18-19de-4e64-a99a-b094689463b6, null, 18ba4c2e-e8d5-4032-a6ab-d0fca3f0f984 RRID:CVCL_A258 KCL025 ["KCL-025", "KCL024_NF1-2"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. no ["cells cannot be used for treatment purposes"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 809313a5-d663-4433-9a89-cb34a350426b Homo sapiens Unknown null null null null 0bf052d2-213f-46fd-8598-ff39c9aacaa9 King's College London https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx null null null null null 8c670bac-17e2-4d75-bc5c-9e5788f293b0 10.1016/j.scr.2016.01.010 ["Heema Hewitson", "Victoria Wood", "Neli Kadeva", "Glenda Cornwell", "Stefano Codognotto", "Emma Stephenson", "Dusko Ilic"] The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays. Stem Cell Research efcb4b18-19de-4e64-a99a-b094689463b6 null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 809313a5-d663-4433-9a89-cb34a350426b, null, null, 33, null, null, null, null, Stem Cell Research, ["general NF1 deficiency"], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, 18ba4c2e-e8d5-4032-a6ab-d0fca3f0f984, KCL025, cellLine, RRID:CVCL_A258, Unknown, Homo sapiens, null, null, ["KCL-025", "KCL024_NF1-2"], null, null, Not Applicable, ["cells cannot be used for treatment purposes"], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]
[null, null, null, null, null, null, Embryonic stem cell, 3a7053c7-05f4-4a29-9957-587d51240a92, null, cc2045df-5ebf-4491-b5b1-88d65e59b228 RRID:CVCL_YL58 SZ-NF2 ["SZ-NF2 PGD", "NF#2"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. yes ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 5b3c4bf5-21a8-4020-8e6f-bed3be6dd7c6 Homo sapiens Unknown null null null null null null null 247530ea-aba9-4560-9c67-39f6f9de532f null Rachel Eiges null https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/ null null null null null 3a7053c7-05f4-4a29-9957-587d51240a92 null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 5b3c4bf5-21a8-4020-8e6f-bed3be6dd7c6, null, null, 21, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, cc2045df-5ebf-4491-b5b1-88d65e59b228, SZ-NF2, cellLine, RRID:CVCL_YL58, Unknown, Homo sapiens, null, null, ["SZ-NF2 PGD", "NF#2"], null, null, Not Applicable, ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 0ded24be-ba48-484f-95b5-4dcb995e708a, null, 09c988ab-765a-44ca-b2d7-1957b729208e RRID:CVCL_YL59 SZ-NF4 ["SZ-NF4 PGD", "NF#4"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. yes ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 1420b3e6-04f9-4e8c-846b-04968327e31f Homo sapiens Unknown null null null null null null null 247530ea-aba9-4560-9c67-39f6f9de532f null Rachel Eiges null https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/ null null null null null 0ded24be-ba48-484f-95b5-4dcb995e708a null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 1420b3e6-04f9-4e8c-846b-04968327e31f, null, null, 22, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, 09c988ab-765a-44ca-b2d7-1957b729208e, SZ-NF4, cellLine, RRID:CVCL_YL59, Unknown, Homo sapiens, null, null, ["SZ-NF4 PGD", "NF#4"], null, null, Not Applicable, ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 7d6c779a-053e-448d-9a61-203d6c72decc, null, cd38ffda-2db1-47f0-af6f-8de572e06037 RRID:CVCL_Y368 Lis42_NF1_1N ["Lis42_NF1_1_N", "Lis 42_NF1_1_N"] cellLine Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1. yes ["none"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 b5b23dce-2d21-4490-b9eb-95c46e2ac0eb Homo sapiens Unknown null null null null null null null 7055567b-7d74-440c-a92e-22a94b795e14 null Dalit Ben Yosef null https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx null null null null null 7d6c779a-053e-448d-9a61-203d6c72decc null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b5b23dce-2d21-4490-b9eb-95c46e2ac0eb, null, null, 24, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, cd38ffda-2db1-47f0-af6f-8de572e06037, Lis42_NF1_1N, cellLine, RRID:CVCL_Y368, Unknown, Homo sapiens, null, null, ["Lis42_NF1_1_N", "Lis 42_NF1_1_N"], null, null, Not Applicable, ["none"], null, null, null, null]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, [Heema Hewitson, Victoria Wood, Neli Kadeva, Glenda Cornwell, Stefano Codognotto, Emma Stephenson, Dusko Ilic], null, null, Embryonic stem cell, 86d1445f-0572-44c0-9301-75da0c1856fd, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 4442c058-074b-4b2e-9bb3-80a1a6cb75d1, null, null, null, null, null, null, Stem Cell Research, [general NF1 deficiency], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, e5fdcfb8-24e7-46fa-9f48-bcbae8a90b7a, KCL024, cellLine, RRID:CVCL_A257, Unknown, Homo sapiens, null, null, [KCL-024, KCL024_NF1-1], null, null, Not Applicable, [cells cannot be used for treatment purposes], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, [Heema Hewitson, Victoria Wood, Neli Kadeva, Glenda Cornwell, Stefano Codognotto, Emma Stephenson, Dusko Ilic], null, null, Embryonic stem cell, efcb4b18-19de-4e64-a99a-b094689463b6, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 809313a5-d663-4433-9a89-cb34a350426b, null, null, null, null, null, null, Stem Cell Research, [general NF1 deficiency], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, 18ba4c2e-e8d5-4032-a6ab-d0fca3f0f984, KCL025, cellLine, RRID:CVCL_A258, Unknown, Homo sapiens, null, null, [KCL-025, KCL024_NF1-2], null, null, Not Applicable, [cells cannot be used for treatment purposes], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]
[null, null, null, null, null, null, Embryonic stem cell, 9fc0cb6e-c5f6-4f08-a9d0-8f5e046659ab, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 4edead03-da70-41fa-829d-2bd511e9f3c1, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, 33cdb482-618e-4489-87d9-3139cc7c6a49, SZ-NF1, cellLine, RRID:CVCL_YL57, Unknown, Homo sapiens, null, null, [SZ-NF1 PGD, NF#1], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 3a7053c7-05f4-4a29-9957-587d51240a92, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 5b3c4bf5-21a8-4020-8e6f-bed3be6dd7c6, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, cc2045df-5ebf-4491-b5b1-88d65e59b228, SZ-NF2, cellLine, RRID:CVCL_YL58, Unknown, Homo sapiens, null, null, [SZ-NF2 PGD, NF#2], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 0ded24be-ba48-484f-95b5-4dcb995e708a, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 1420b3e6-04f9-4e8c-846b-04968327e31f, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, 09c988ab-765a-44ca-b2d7-1957b729208e, SZ-NF4, cellLine, RRID:CVCL_YL59, Unknown, Homo sapiens, null, null, [SZ-NF4 PGD, NF#4], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 7d6c779a-053e-448d-9a61-203d6c72decc, null, Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b5b23dce-2d21-4490-b9eb-95c46e2ac0eb, null, null, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, cd38ffda-2db1-47f0-af6f-8de572e06037, Lis42_NF1_1N, cellLine, RRID:CVCL_Y368, Unknown, Homo sapiens, null, null, [Lis42_NF1_1_N, Lis 42_NF1_1_N], null, null, Not Applicable, [none], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 4bd71d55-44c3-4a17-b921-5510932b9d1e, null, Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b6f8726c-3d3a-43ec-a2c3-46d4c56cf06e, null, null, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, d0ca95f1-a3d9-4641-b47d-84346d9ec04a, Lis47_NF1_2N, cellLine, RRID:CVCL_Y373, Unknown, Homo sapiens, null, null, [Lis47_NF1_2_N, Lis 47_NF1_1_N], null, null, Not Applicable, [none], null, null, null, null]
[null, null, null, null, 00301-00122, null, Cancer cell line, 3d91f1a4-8a12-4566-af2a-37e895964ebc, null, HeLa cervical cancer cells with stable (EBV-based siRNA) knockdown of the NF1 gene., Neurofibromatosis type 1, null, 418d199d-c37a-4695-acb7-807122379fcb, null, null, null, null, null, null, null, [cervical adenocarcinoma], unknown, null, null, null, null, null, Black, null, 2549e536-b033-43e4-acf6-501499b6e498, HeLa SilenciX NF1, cellLine, RRID:CVCL_KT82, Female, Homo sapiens, null, null, [NF1 HeLa SilenciX], null, null, cervical adenocarcinoma, [unknown], ef31e013-9a9e-45aa-8066-af8680c0115e, ef31e013-9a9e-45aa-8066-af8680c0115e, tebu-bio, https://www.tebu-bio.com]
[Human neurofibromatosis type 1 is a dominant disease caused by the inheritance of a mutant allele of the NF1 gene. In order to study NF1 function, we have constructed a mouse strain carrying a germline mutation in the murine homologue. Heterozygous animals do not exhibit the classical symptoms of the human disease, but are highly predisposed to the formation of various tumour types, notably phaeochomocytoma, a tumour of the neural crest-derived adrenal medulla, and myeloid leukaemia, both of which occur with increased frequency in human NF1 patients. The wild-type Nf1 allele is lost in approximately half of the tumours from heterozygous animals. In addition, homozygosity for the Nf1 mutation leads to abnormal cardiac development and mid-gestational embryonic lethality., c9ccb5f0-587d-4c2c-ba8b-184013b41d44, mouse cells, [T Jacks, T S Shih, E M Schmitt, R T Bronson, A Bernards, R A Weinberg], JAX_008192, https://www.jax.org/strain/008192, null, null, null, [From JAX]: Heterozygous animals do not exhibit the classical symptoms of Human neurofibromatosis type 1, but are highly predisposed to the formation of various tumor types, notably phaeochromocytoma, a tumor of the neural crest-derived adrenal medulla, and myeloid leukemia. Homozygosity leads to abnormal cardiac development and mid-gestational embryonic lethality. This strain may be useful in studies of cancer and developmental biology., Neurofibromatosis type 1, 10.1038/ng0794-353, 7b50b518-1d02-4186-a701-733abd204b78, null, C57BL/6, Massachusetts Institute of Technology, bba58be3-c41e-4d53-bf4e-7cd9dbbecc0e, Tyler Jacks, null, Nature Genetics, [pheochromocytoma, acute myeloid leukemia, tumor of the neural crest-derived adrenal medulla], yes, null, null, null, null, 09f4d299-2d0c-4831-82a5-eae2ef3229d2, null, null, 45638793-f2d0-4c40-8b1c-be1f1b0c0f93, B6.129S2-Nf1tm1Tyj/J, animalModel, RRID:IMSR_JAX:008192, null, Mus musculus, null, B6.129S2-Nf1tm1Tyj/J, null, null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, 81042b9d-3d66-45c8-9c7d-8b0c90bb4b6c, The Jackson Laboratory, https://www.jax.org]
[Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology. Introduction of a Synapsin I promoter driven Cre transgenic mouse strain into the conditional NF1 background has ablated NF1 function in most differentiated neuronal populations. These mice have abnormal development of the cerebral cortex, which suggests that NF1 has an indispensable role in this aspect of CNS development. Furthermore, although they are tumor free, these mice display extensive astrogliosis in the absence of conspicuous neurodegeneration or microgliosis. These results indicate that NF1-deficient neurons are capable of inducing reactive astrogliosis via a non-cell autonomous mechanism., b75c336e-791d-4a62-b556-153c88b28960, mouse cells, [Y Zhu, M I Romero, P Ghosh, Z Ye, P Charnay, E J Rushing, J D Marth, L F Parada], JAX_017640, https://www.jax.org/strain/017640, null, null, null, [From JAX]: These mice possess loxP sites flanking exons 31-32 of the neurofibromatosis 1 (Nf1) gene and have applications in studies of cancer, neural crest development and neurofibromatosis type I., Neurofibromatosis type 1, 10.1101/gad.862101, be180150-374d-4459-9a81-398c8a34bca9, null, C57BL/6J, UT Southwestern Medical Center, bb909638-4123-41a1-bd61-b300c13e7652, Luis Parada, null, Genes & Development, [plexiform neurofibroma], yes, null, null, null, null, b0332348-464f-4952-a7f7-2ffe4dc6b3f9, null, null, 118aa95e-27f4-48fa-94c7-5f786b8b4f2f, B6.129(Cg)-Nf1tm1Par/J, animalModel, RRID:IMSR_JAX:017640, null, Mus musculus, null, B6.129(Cg)-Nf1tm1Par/J, [Nf1flox], null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, f137e564-d395-4226-bc60-149c3c760e99, The Jackson Laboratory, https://www.jax.org]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, [Heema Hewitson, Victoria Wood, Neli Kadeva, Glenda Cornwell, Stefano Codognotto, Emma Stephenson, Dusko Ilic], null, null, Embryonic stem cell, 86d1445f-0572-44c0-9301-75da0c1856fd, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 4442c058-074b-4b2e-9bb3-80a1a6cb75d1, null, null, null, null, null, null, Stem Cell Research, [general NF1 deficiency], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, e5fdcfb8-24e7-46fa-9f48-bcbae8a90b7a, KCL024, cellLine, RRID:CVCL_A257, Unknown, Homo sapiens, null, null, [KCL-024, KCL024_NF1-1], null, null, Not Applicable, [cells cannot be used for treatment purposes], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, [Heema Hewitson, Victoria Wood, Neli Kadeva, Glenda Cornwell, Stefano Codognotto, Emma Stephenson, Dusko Ilic], null, null, Embryonic stem cell, efcb4b18-19de-4e64-a99a-b094689463b6, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 809313a5-d663-4433-9a89-cb34a350426b, null, null, null, null, null, null, Stem Cell Research, [general NF1 deficiency], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, 18ba4c2e-e8d5-4032-a6ab-d0fca3f0f984, KCL025, cellLine, RRID:CVCL_A258, Unknown, Homo sapiens, null, null, [KCL-025, KCL024_NF1-2], null, null, Not Applicable, [cells cannot be used for treatment purposes], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]
[null, null, null, null, null, null, Embryonic stem cell, 9fc0cb6e-c5f6-4f08-a9d0-8f5e046659ab, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 4edead03-da70-41fa-829d-2bd511e9f3c1, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, 33cdb482-618e-4489-87d9-3139cc7c6a49, SZ-NF1, cellLine, RRID:CVCL_YL57, Unknown, Homo sapiens, null, null, [SZ-NF1 PGD, NF#1], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 3a7053c7-05f4-4a29-9957-587d51240a92, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 5b3c4bf5-21a8-4020-8e6f-bed3be6dd7c6, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, cc2045df-5ebf-4491-b5b1-88d65e59b228, SZ-NF2, cellLine, RRID:CVCL_YL58, Unknown, Homo sapiens, null, null, [SZ-NF2 PGD, NF#2], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 0ded24be-ba48-484f-95b5-4dcb995e708a, null, Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 1420b3e6-04f9-4e8c-846b-04968327e31f, null, null, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, 09c988ab-765a-44ca-b2d7-1957b729208e, SZ-NF4, cellLine, RRID:CVCL_YL59, Unknown, Homo sapiens, null, null, [SZ-NF4 PGD, NF#4], null, null, Not Applicable, [non-commercial use only, generation of germ line transmitting chimeras not permitted, creation of functional gametes not permitted, generation of interspecies chimeras not permitted, human cloning not permitted, IRB documentation required], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 7d6c779a-053e-448d-9a61-203d6c72decc, null, Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b5b23dce-2d21-4490-b9eb-95c46e2ac0eb, null, null, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, cd38ffda-2db1-47f0-af6f-8de572e06037, Lis42_NF1_1N, cellLine, RRID:CVCL_Y368, Unknown, Homo sapiens, null, null, [Lis42_NF1_1_N, Lis 42_NF1_1_N], null, null, Not Applicable, [none], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 4bd71d55-44c3-4a17-b921-5510932b9d1e, null, Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b6f8726c-3d3a-43ec-a2c3-46d4c56cf06e, null, null, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, [general NF1 deficiency], yes, null, null, null, null, null, null, null, d0ca95f1-a3d9-4641-b47d-84346d9ec04a, Lis47_NF1_2N, cellLine, RRID:CVCL_Y373, Unknown, Homo sapiens, null, null, [Lis47_NF1_2_N, Lis 47_NF1_1_N], null, null, Not Applicable, [none], null, null, null, null]
[Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology. Introduction of a Synapsin I promoter driven Cre transgenic mouse strain into the conditional NF1 background has ablated NF1 function in most differentiated neuronal populations. These mice have abnormal development of the cerebral cortex, which suggests that NF1 has an indispensable role in this aspect of CNS development. Furthermore, although they are tumor free, these mice display extensive astrogliosis in the absence of conspicuous neurodegeneration or microgliosis. These results indicate that NF1-deficient neurons are capable of inducing reactive astrogliosis via a non-cell autonomous mechanism., b75c336e-791d-4a62-b556-153c88b28960, mouse cells, [Y Zhu, M I Romero, P Ghosh, Z Ye, P Charnay, E J Rushing, J D Marth, L F Parada], JAX_017640, https://www.jax.org/strain/017640, null, null, null, [From JAX]: These mice possess loxP sites flanking exons 31-32 of the neurofibromatosis 1 (Nf1) gene and have applications in studies of cancer, neural crest development and neurofibromatosis type I., Neurofibromatosis type 1, 10.1101/gad.862101, be180150-374d-4459-9a81-398c8a34bca9, null, C57BL/6J, UT Southwestern Medical Center, bb909638-4123-41a1-bd61-b300c13e7652, Luis Parada, null, Genes & Development, [plexiform neurofibroma], yes, null, null, null, null, b0332348-464f-4952-a7f7-2ffe4dc6b3f9, null, null, 118aa95e-27f4-48fa-94c7-5f786b8b4f2f, B6.129(Cg)-Nf1tm1Par/J, animalModel, RRID:IMSR_JAX:017640, null, Mus musculus, null, B6.129(Cg)-Nf1tm1Par/J, [Nf1flox], null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, f137e564-d395-4226-bc60-149c3c760e99, The Jackson Laboratory, https://www.jax.org]
[Human neurofibromatosis type 1 is a dominant disease caused by the inheritance of a mutant allele of the NF1 gene. In order to study NF1 function, we have constructed a mouse strain carrying a germline mutation in the murine homologue. Heterozygous animals do not exhibit the classical symptoms of the human disease, but are highly predisposed to the formation of various tumour types, notably phaeochomocytoma, a tumour of the neural crest-derived adrenal medulla, and myeloid leukaemia, both of which occur with increased frequency in human NF1 patients. The wild-type Nf1 allele is lost in approximately half of the tumours from heterozygous animals. In addition, homozygosity for the Nf1 mutation leads to abnormal cardiac development and mid-gestational embryonic lethality., c9ccb5f0-587d-4c2c-ba8b-184013b41d44, mouse cells, [T Jacks, T S Shih, E M Schmitt, R T Bronson, A Bernards, R A Weinberg], JAX_008192, https://www.jax.org/strain/008192, null, null, null, [From JAX]: Heterozygous animals do not exhibit the classical symptoms of Human neurofibromatosis type 1, but are highly predisposed to the formation of various tumor types, notably phaeochromocytoma, a tumor of the neural crest-derived adrenal medulla, and myeloid leukemia. Homozygosity leads to abnormal cardiac development and mid-gestational embryonic lethality. This strain may be useful in studies of cancer and developmental biology., Neurofibromatosis type 1, 10.1038/ng0794-353, 7b50b518-1d02-4186-a701-733abd204b78, null, C57BL/6, Massachusetts Institute of Technology, bba58be3-c41e-4d53-bf4e-7cd9dbbecc0e, Tyler Jacks, null, Nature Genetics, [pheochromocytoma, acute myeloid leukemia, tumor of the neural crest-derived adrenal medulla], yes, null, null, null, null, 09f4d299-2d0c-4831-82a5-eae2ef3229d2, null, null, 45638793-f2d0-4c40-8b1c-be1f1b0c0f93, B6.129S2-Nf1tm1Tyj/J, animalModel, RRID:IMSR_JAX:008192, null, Mus musculus, null, B6.129S2-Nf1tm1Tyj/J, null, null, null, null, [Unknown], a3d45625-7ff1-4a33-a67b-af0736412f6c, 81042b9d-3d66-45c8-9c7d-8b0c90bb4b6c, The Jackson Laboratory, https://www.jax.org]
[null, null, null, null, JCRB3015, null, Finite cell line, 50ca52b3-8448-4e82-a9ee-148d6cda19f1, null, Cell line from an NF1 patient; unclear if derived from tumor or non-tumor tissue., Neurofibromatosis type 1, null, f860ef0a-2501-4fa1-b0be-f638bde25c6d, null, null, null, null, null, null, null, [general NF1 deficiency], unknown, null, null, null, null, null, Asian, null, 1a8a362e-e3be-4bc3-be7e-d0a0865b9c31, NF1, cellLine, RRID:CVCL_JG80, Male, Homo sapiens, null, null, null, null, null, Not Applicable, [unknown], 247530ea-aba9-4560-9c67-39f6f9de532f, 111541ac-94a0-4190-babc-cf5be4bbd1ab, JCRB Cell Bank, https://cellbank.nibiohn.go.jp]

[ABSTRACT, ANIMALMODELID, ANIMALSTATE, AUTHORS, CATALOGNUMBER, CATALOGNUMBERURL, CELLLINECATEGORY, CELLLINEID, CONTAMINATEDMISIDENTIFIED, CONTENT_TEXT, DESCRIPTION, DISEASE, DOI, DONORID, GENERATION, GENETICBACKGROUND, ID, INSTITUTION, INVESTIGATORID, INVESTIGATORNAME, INVESTIGATORWEBSITE, JOURNAL, MODELOFMANIFESTATION, MTAREQUIRED, ORCID, ORGAN, ORIGINYEAR, POPULATIONDOUBLINGTIME, PUBLICATIONID, RACE, RESISTANCE, RESOURCEID, RESOURCENAME, RESOURCETYPE, RRID, SEX, SPECIES, STRAINNOMENCLATURE, STRPROFILE, SYNONYMS, TISSUE, TRANSPLANTATIONTYPE, TUMORTYPE, USAGERESTRICTIONS, VENDORID, VENDORITEMID, VENDORNAME, WEBSITE]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, ["Heema Hewitson", "Victoria Wood", "Neli Kadeva", "Glenda Cornwell", "Stefano Codognotto", "Emma Stephenson", "Dusko Ilic"], null, null, Embryonic stem cell, 86d1445f-0572-44c0-9301-75da0c1856fd, null, e5fdcfb8-24e7-46fa-9f48-bcbae8a90b7a RRID:CVCL_A257 KCL024 ["KCL-024", "KCL024_NF1-1"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. no ["cells cannot be used for treatment purposes"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4442c058-074b-4b2e-9bb3-80a1a6cb75d1 Homo sapiens Unknown null null null null 0bf052d2-213f-46fd-8598-ff39c9aacaa9 King's College London https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx null null null null null 8c670bac-17e2-4d75-bc5c-9e5788f293b0 10.1016/j.scr.2016.01.010 ["Heema Hewitson", "Victoria Wood", "Neli Kadeva", "Glenda Cornwell", "Stefano Codognotto", "Emma Stephenson", "Dusko Ilic"] The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays. Stem Cell Research 86d1445f-0572-44c0-9301-75da0c1856fd null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 4442c058-074b-4b2e-9bb3-80a1a6cb75d1, null, null, 32, null, null, null, null, Stem Cell Research, ["general NF1 deficiency"], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, e5fdcfb8-24e7-46fa-9f48-bcbae8a90b7a, KCL024, cellLine, RRID:CVCL_A257, Unknown, Homo sapiens, null, null, ["KCL-024", "KCL024_NF1-1"], null, null, Not Applicable, ["cells cannot be used for treatment purposes"], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]
[The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays., null, null, ["Heema Hewitson", "Victoria Wood", "Neli Kadeva", "Glenda Cornwell", "Stefano Codognotto", "Emma Stephenson", "Dusko Ilic"], null, null, Embryonic stem cell, efcb4b18-19de-4e64-a99a-b094689463b6, null, 18ba4c2e-e8d5-4032-a6ab-d0fca3f0f984 RRID:CVCL_A258 KCL025 ["KCL-025", "KCL024_NF1-2"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. no ["cells cannot be used for treatment purposes"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 809313a5-d663-4433-9a89-cb34a350426b Homo sapiens Unknown null null null null 0bf052d2-213f-46fd-8598-ff39c9aacaa9 King's College London https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx null null null null null 8c670bac-17e2-4d75-bc5c-9e5788f293b0 10.1016/j.scr.2016.01.010 ["Heema Hewitson", "Victoria Wood", "Neli Kadeva", "Glenda Cornwell", "Stefano Codognotto", "Emma Stephenson", "Dusko Ilic"] The KCL024 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation in the NF1 gene encoding neurofibromin (c.3739-3742 ∆TTTG). Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment. Pluripotent state and differentiation potential were confirmed by in vitro assays. Stem Cell Research efcb4b18-19de-4e64-a99a-b094689463b6 null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, 10.1016/j.scr.2016.01.010, 809313a5-d663-4433-9a89-cb34a350426b, null, null, 33, null, null, null, null, Stem Cell Research, ["general NF1 deficiency"], no, null, null, null, null, 8c670bac-17e2-4d75-bc5c-9e5788f293b0, null, null, 18ba4c2e-e8d5-4032-a6ab-d0fca3f0f984, KCL025, cellLine, RRID:CVCL_A258, Unknown, Homo sapiens, null, null, ["KCL-025", "KCL024_NF1-2"], null, null, Not Applicable, ["cells cannot be used for treatment purposes"], 0bf052d2-213f-46fd-8598-ff39c9aacaa9, null, King's College London, https://www.kcl.ac.uk/lsm/research/divisions/wh/groups/medicine/hescell.aspx]
[null, null, null, null, null, null, Embryonic stem cell, 7d6c779a-053e-448d-9a61-203d6c72decc, null, cd38ffda-2db1-47f0-af6f-8de572e06037 RRID:CVCL_Y368 Lis42_NF1_1N ["Lis42_NF1_1_N", "Lis 42_NF1_1_N"] cellLine Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1. yes ["none"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 b5b23dce-2d21-4490-b9eb-95c46e2ac0eb Homo sapiens Unknown null null null null null null null 7055567b-7d74-440c-a92e-22a94b795e14 null Dalit Ben Yosef null https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx null null null null null 7d6c779a-053e-448d-9a61-203d6c72decc null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b5b23dce-2d21-4490-b9eb-95c46e2ac0eb, null, null, 24, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, cd38ffda-2db1-47f0-af6f-8de572e06037, Lis42_NF1_1N, cellLine, RRID:CVCL_Y368, Unknown, Homo sapiens, null, null, ["Lis42_NF1_1_N", "Lis 42_NF1_1_N"], null, null, Not Applicable, ["none"], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 4bd71d55-44c3-4a17-b921-5510932b9d1e, null, d0ca95f1-a3d9-4641-b47d-84346d9ec04a RRID:CVCL_Y373 Lis47_NF1_2N ["Lis47_NF1_2_N", "Lis 47_NF1_1_N"] cellLine Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1. yes ["none"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 b6f8726c-3d3a-43ec-a2c3-46d4c56cf06e Homo sapiens Unknown null null null null null null null 7055567b-7d74-440c-a92e-22a94b795e14 null Dalit Ben Yosef null https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx null null null null null 4bd71d55-44c3-4a17-b921-5510932b9d1e null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with neurofibromatosis type 1., Neurofibromatosis type 1, null, b6f8726c-3d3a-43ec-a2c3-46d4c56cf06e, null, null, 25, null, 7055567b-7d74-440c-a92e-22a94b795e14, Dalit Ben Yosef, https://www.tasmc.org.il/sites/en/Research/Tech-Transfer/Stem-Cell-Research-laboratory/Pages/Stem-Cell-Research-laboratory.aspx, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, d0ca95f1-a3d9-4641-b47d-84346d9ec04a, Lis47_NF1_2N, cellLine, RRID:CVCL_Y373, Unknown, Homo sapiens, null, null, ["Lis47_NF1_2_N", "Lis 47_NF1_1_N"], null, null, Not Applicable, ["none"], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 9fc0cb6e-c5f6-4f08-a9d0-8f5e046659ab, null, 33cdb482-618e-4489-87d9-3139cc7c6a49 RRID:CVCL_YL57 SZ-NF1 ["SZ-NF1 PGD", "NF#1"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. yes ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 4edead03-da70-41fa-829d-2bd511e9f3c1 Homo sapiens Unknown null null null null null null null 247530ea-aba9-4560-9c67-39f6f9de532f null Rachel Eiges null https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/ null null null null null 9fc0cb6e-c5f6-4f08-a9d0-8f5e046659ab null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 4edead03-da70-41fa-829d-2bd511e9f3c1, null, null, 20, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, 33cdb482-618e-4489-87d9-3139cc7c6a49, SZ-NF1, cellLine, RRID:CVCL_YL57, Unknown, Homo sapiens, null, null, ["SZ-NF1 PGD", "NF#1"], null, null, Not Applicable, ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 3a7053c7-05f4-4a29-9957-587d51240a92, null, cc2045df-5ebf-4491-b5b1-88d65e59b228 RRID:CVCL_YL58 SZ-NF2 ["SZ-NF2 PGD", "NF#2"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. yes ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 5b3c4bf5-21a8-4020-8e6f-bed3be6dd7c6 Homo sapiens Unknown null null null null null null null 247530ea-aba9-4560-9c67-39f6f9de532f null Rachel Eiges null https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/ null null null null null 3a7053c7-05f4-4a29-9957-587d51240a92 null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 5b3c4bf5-21a8-4020-8e6f-bed3be6dd7c6, null, null, 21, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, cc2045df-5ebf-4491-b5b1-88d65e59b228, SZ-NF2, cellLine, RRID:CVCL_YL58, Unknown, Homo sapiens, null, null, ["SZ-NF2 PGD", "NF#2"], null, null, Not Applicable, ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"], null, null, null, null]
[null, null, null, null, null, null, Embryonic stem cell, 0ded24be-ba48-484f-95b5-4dcb995e708a, null, 09c988ab-765a-44ca-b2d7-1957b729208e RRID:CVCL_YL59 SZ-NF4 ["SZ-NF4 PGD", "NF#4"] cellLine Human embryonic stem cell line derived from an embryo with an NF1 mutation. yes ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"] null null null null null null ["general NF1 deficiency"] Neurofibromatosis type 1 1420b3e6-04f9-4e8c-846b-04968327e31f Homo sapiens Unknown null null null null null null null 247530ea-aba9-4560-9c67-39f6f9de532f null Rachel Eiges null https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/ null null null null null 0ded24be-ba48-484f-95b5-4dcb995e708a null null Embryonic stem cell null null null null null Not Applicable , Human embryonic stem cell line derived from an embryo with an NF1 mutation., Neurofibromatosis type 1, null, 1420b3e6-04f9-4e8c-846b-04968327e31f, null, null, 22, null, 247530ea-aba9-4560-9c67-39f6f9de532f, Rachel Eiges, https://www.szmc.org.il/eng/Departments/Stem-Cell-Research-Laboratory/About/, null, ["general NF1 deficiency"], yes, null, null, null, null, null, null, null, 09c988ab-765a-44ca-b2d7-1957b729208e, SZ-NF4, cellLine, RRID:CVCL_YL59, Unknown, Homo sapiens, null, null, ["SZ-NF4 PGD", "NF#4"], null, null, Not Applicable, ["non-commercial use only", "generation of germ line transmitting chimeras not permitted", "creation of functional gametes not permitted", "generation of interspecies chimeras not permitted", "human cloning not permitted", "IRB documentation required"], null, null, null, null]
[Human neurofibromatosis type 1 is a dominant disease caused by the inheritance of a mutant allele of the NF1 gene. In order to study NF1 function, we have constructed a mouse strain carrying a germline mutation in the murine homologue. Heterozygous animals do not exhibit the classical symptoms of the human disease, but are highly predisposed to the formation of various tumour types, notably phaeochomocytoma, a tumour of the neural crest-derived adrenal medulla, and myeloid leukaemia, both of which occur with increased frequency in human NF1 patients. The wild-type Nf1 allele is lost in approximately half of the tumours from heterozygous animals. In addition, homozygosity for the Nf1 mutation leads to abnormal cardiac development and mid-gestational embryonic lethality., c9ccb5f0-587d-4c2c-ba8b-184013b41d44, mouse cells, ["T Jacks", "T S Shih", "E M Schmitt", "R T Bronson", "A Bernards", "R A Weinberg"], JAX_008192, https://www.jax.org/strain/008192, null, null, null, 45638793-f2d0-4c40-8b1c-be1f1b0c0f93 RRID:IMSR_JAX:008192 B6.129S2-Nf1tm1Tyj/J null animalModel [From JAX]: Heterozygous animals do not exhibit the classical symptoms of Human neurofibromatosis type 1, but are highly predisposed to the formation of various tumor types, notably phaeochromocytoma, a tumor of the neural crest-derived adrenal medulla, and myeloid leukemia. Homozygosity leads to abnormal cardiac development and mid-gestational embryonic lethality. This strain may be useful in studies of cancer and developmental biology. yes ["Unknown"] c9ccb5f0-587d-4c2c-ba8b-184013b41d44 mouse cells C57BL/6 B6.129S2-Nf1tm1Tyj/J null null ["pheochromocytoma", "acute myeloid leukemia", "tumor of the neural crest-derived adrenal medulla"] Neurofibromatosis type 1 7b50b518-1d02-4186-a701-733abd204b78 Mus musculus null null 81042b9d-3d66-45c8-9c7d-8b0c90bb4b6c JAX_008192 https://www.jax.org/strain/008192 a3d45625-7ff1-4a33-a67b-af0736412f6c The Jackson Laboratory https://www.jax.org bba58be3-c41e-4d53-bf4e-7cd9dbbecc0e null Tyler Jacks Massachusetts Institute of Technology null 09f4d299-2d0c-4831-82a5-eae2ef3229d2 10.1038/ng0794-353 ["T Jacks", "T S Shih", "E M Schmitt", "R T Bronson", "A Bernards", "R A Weinberg"] Human neurofibromatosis type 1 is a dominant disease caused by the inheritance of a mutant allele of the NF1 gene. In order to study NF1 function, we have constructed a mouse strain carrying a germline mutation in the murine homologue. Heterozygous animals do not exhibit the classical symptoms of the human disease, but are highly predisposed to the formation of various tumour types, notably phaeochomocytoma, a tumour of the neural crest-derived adrenal medulla, and myeloid leukaemia, both of which occur with increased frequency in human NF1 patients. The wild-type Nf1 allele is lost in approximately half of the tumours from heterozygous animals. In addition, homozygosity for the Nf1 mutation leads to abnormal cardiac development and mid-gestational embryonic lethality. Nature Genetics null null null null null null null null null null , [From JAX]: Heterozygous animals do not exhibit the classical symptoms of Human neurofibromatosis type 1, but are highly predisposed to the formation of various tumor types, notably phaeochromocytoma, a tumor of the neural crest-derived adrenal medulla, and myeloid leukemia. Homozygosity leads to abnormal cardiac development and mid-gestational embryonic lethality. This strain may be useful in studies of cancer and developmental biology., Neurofibromatosis type 1, 10.1038/ng0794-353, 7b50b518-1d02-4186-a701-733abd204b78, null, C57BL/6, 3, Massachusetts Institute of Technology, bba58be3-c41e-4d53-bf4e-7cd9dbbecc0e, Tyler Jacks, null, Nature Genetics, ["pheochromocytoma", "acute myeloid leukemia", "tumor of the neural crest-derived adrenal medulla"], yes, null, null, null, null, 09f4d299-2d0c-4831-82a5-eae2ef3229d2, null, null, 45638793-f2d0-4c40-8b1c-be1f1b0c0f93, B6.129S2-Nf1tm1Tyj/J, animalModel, RRID:IMSR_JAX:008192, null, Mus musculus, B6.129S2-Nf1tm1Tyj/J, null, null, null, null, null, ["Unknown"], a3d45625-7ff1-4a33-a67b-af0736412f6c, 81042b9d-3d66-45c8-9c7d-8b0c90bb4b6c, The Jackson Laboratory, https://www.jax.org]
[Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology. Introduction of a Synapsin I promoter driven Cre transgenic mouse strain into the conditional NF1 background has ablated NF1 function in most differentiated neuronal populations. These mice have abnormal development of the cerebral cortex, which suggests that NF1 has an indispensable role in this aspect of CNS development. Furthermore, although they are tumor free, these mice display extensive astrogliosis in the absence of conspicuous neurodegeneration or microgliosis. These results indicate that NF1-deficient neurons are capable of inducing reactive astrogliosis via a non-cell autonomous mechanism., b75c336e-791d-4a62-b556-153c88b28960, mouse cells, ["Y Zhu", "M I Romero", "P Ghosh", "Z Ye", "P Charnay", "E J Rushing", "J D Marth", "L F Parada"], JAX_017640, https://www.jax.org/strain/017640, null, null, null, 118aa95e-27f4-48fa-94c7-5f786b8b4f2f RRID:IMSR_JAX:017640 B6.129(Cg)-Nf1tm1Par/J ["Nf1flox"] animalModel [From JAX]: These mice possess loxP sites flanking exons 31-32 of the neurofibromatosis 1 (Nf1) gene and have applications in studies of cancer, neural crest development and neurofibromatosis type I. yes ["Unknown"] b75c336e-791d-4a62-b556-153c88b28960 mouse cells C57BL/6J B6.129(Cg)-Nf1tm1Par/J null null ["plexiform neurofibroma"] Neurofibromatosis type 1 be180150-374d-4459-9a81-398c8a34bca9 Mus musculus null null f137e564-d395-4226-bc60-149c3c760e99 JAX_017640 https://www.jax.org/strain/017640 a3d45625-7ff1-4a33-a67b-af0736412f6c The Jackson Laboratory https://www.jax.org bb909638-4123-41a1-bd61-b300c13e7652 null Luis Parada UT Southwestern Medical Center null b0332348-464f-4952-a7f7-2ffe4dc6b3f9 10.1101/gad.862101 ["Y Zhu", "M I Romero", "P Ghosh", "Z Ye", "P Charnay", "E J Rushing", "J D Marth", "L F Parada"] Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology. Introduction of a Synapsin I promoter driven Cre transgenic mouse strain into the conditional NF1 background has ablated NF1 function in most differentiated neuronal populations. These mice have abnormal development of the cerebral cortex, which suggests that NF1 has an indispensable role in this aspect of CNS development. Furthermore, although they are tumor free, these mice display extensive astrogliosis in the absence of conspicuous neurodegeneration or microgliosis. These results indicate that NF1-deficient neurons are capable of inducing reactive astrogliosis via a non-cell autonomous mechanism. Genes & Development null null null null null null null null null null , [From JAX]: These mice possess loxP sites flanking exons 31-32 of the neurofibromatosis 1 (Nf1) gene and have applications in studies of cancer, neural crest development and neurofibromatosis type I., Neurofibromatosis type 1, 10.1101/gad.862101, be180150-374d-4459-9a81-398c8a34bca9, null, C57BL/6J, 5, UT Southwestern Medical Center, bb909638-4123-41a1-bd61-b300c13e7652, Luis Parada, null, Genes & Development, ["plexiform neurofibroma"], yes, null, null, null, null, b0332348-464f-4952-a7f7-2ffe4dc6b3f9, null, null, 118aa95e-27f4-48fa-94c7-5f786b8b4f2f, B6.129(Cg)-Nf1tm1Par/J, animalModel, RRID:IMSR_JAX:017640, null, Mus musculus, B6.129(Cg)-Nf1tm1Par/J, null, ["Nf1flox"], null, null, null, ["Unknown"], a3d45625-7ff1-4a33-a67b-af0736412f6c, f137e564-d395-4226-bc60-149c3c760e99, The Jackson Laboratory, https://www.jax.org]
[null, null, null, null, 5PNF_TDiPSsv_PM_6, null, Induced pluripotent stem cell, 9d07a958-d8e8-447b-a337-d0be0ee206e5, null, 8ebe9be8-88df-4598-bd25-510de550ca5e RRID:CVCL_UN14 5PNF_TDiPSsv_PM_6 null cellLine Cutaneous neurofibroma-derived iPSC banked in the Barcelona node of the Spanish National Cell Bank. unknown ["unknown"] null null null null null null ["cutaneous neurofibroma"] Neurofibromatosis type 1 fd6e6f02-bf0f-48db-b1ad-9696630bed4d Homo sapiens Male null 4d9f670f-485c-439d-8be9-7418438950a7 5PNF_TDiPSsv_PM_6 null 1821d1da-302e-4380-adb2-fc54809fbf6d BNLC https://www.isciii.es/QueHacemos/Servicios/BIOBANCOS/BNLC/Paginas/LineasiPS.aspx null null null null null null null null null null 9d07a958-d8e8-447b-a337-d0be0ee206e5 null null Induced pluripotent stem cell null null null null null cutaneous neurofibroma , Cutaneous neurofibroma-derived iPSC banked in the Barcelona node of the Spanish National Cell Bank., Neurofibromatosis type 1, null, fd6e6f02-bf0f-48db-b1ad-9696630bed4d, null, null, 34, null, null, null, null, null, ["cutaneous neurofibroma"], unknown, null, null, null, null, null, null, null, 8ebe9be8-88df-4598-bd25-510de550ca5e, 5PNF_TDiPSsv_PM_6, cellLine, RRID:CVCL_UN14, Male, Homo sapiens, null, null, null, null, null, cutaneous neurofibroma, ["unknown"], 1821d1da-302e-4380-adb2-fc54809fbf6d, 4d9f670f-485c-439d-8be9-7418438950a7, BNLC, https://www.isciii.es/QueHacemos/Servicios/BIOBANCOS/BNLC/Paginas/LineasiPS.aspx]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[Skin fibroblast cell strains and tumour cell lines were established from 12 patients with various types of soft tissue neoplasms, and radiation survival curve parameters were measured in vitro. Soft tissue sarcoma cells were consistently more sensitive to X-irradiation than fibroblasts isolated from the same patient, and were also more sensitive as a group than cell lines derived from 34 other human tumours. There was a general correlation in radiosensitivity between fibroblasts and tumour cells derived from the same patient, indicating that some component of tumour cell sensitivity may relate to genetic factors in the host. Such genetic factors, however, do not explain all of the heterogeneity in tumour cell response. The response of soft tissue sarcoma in vivo may be dependent on complex radiomodifying factors other than inherent radiation sensitivity, thus making it difficult to predict clinical outcome by use of assays which use survival of irradiated tumour cell lines in vitro as an endpoint., null, null, [W K Dahlberg, J B Little, J A Fletcher, H D Suit, P Okunieff], null, null, Cancer cell line, 1139e45c-9645-449f-a22e-7497cbffb0b3, null, Sporadic MPNST tumor cell line from a non-NF1 patient., Cancer, 10.1080/09553009314550251, b50e7512-a686-4c50-9e95-6af9d06bf826, null, null, null, null, null, null, International Journal of Radiation Biology, [malignant peripheral nerve sheath tumor], unknown, null, null, null, null, 979d13af-cd42-4fcf-ad7f-bd52e4b28bcf, null, null, 9a7997c9-9399-47ed-b44b-5b717be89ba3, STS-26T, cellLine, RRID:CVCL_8917, Female, Homo sapiens, null, null, [STS26, STS26T], null, null, malignant peripheral nerve sheath tumor, [unknown], null, null, null, null]
[null, null, null, null, GM11602, null, Transformed cell line, a76cad86-d2a2-402d-bb06-5ee13a8cdcec, null, Leukemia cell line derived from B-lymphocytes from an NF1 patient., Neurofibromatosis type 1, null, 49b6cb1f-7231-4208-a012-41c9bffff993, null, null, null, null, null, null, null, [acute lymphocytic leukemia], unknown, null, null, null, null, null, White, null, f354ec1a-0305-4e6c-897a-10e4fba10a28, GM11602, cellLine, RRID:CVCL_AA02, Female, Homo sapiens, null, null, null, null, null, acute lymphocytic leukemia, [unknown], 6c22c677-407b-463c-b53c-6dfa3c50e681, 56da4429-da43-44c8-862b-8a70955091df, Corielle Institute, https://www.coriell.org]
[null, null, null, null, GM11601, null, Transformed cell line, 58d36942-062c-4db1-91f9-ee8cda50f4fc, null, Leukemia cell line derived from B-lymphocytes from an NF1 patient., Neurofibromatosis type 1, null, 0460814f-4395-46bd-8acf-f1ded8afde9d, null, null, null, null, null, null, null, [acute lymphocytic leukemia], unknown, null, null, null, null, null, White, null, a2f62c57-a1b8-4867-9c91-04406f261cfa, GM11601, cellLine, RRID:CVCL_AA01, Male, Homo sapiens, null, null, null, null, null, acute lymphocytic leukemia, [unknown], 6c22c677-407b-463c-b53c-6dfa3c50e681, 495b8f81-36bc-4273-8e78-27ed87ef48c1, Corielle Institute, https://www.coriell.org]
[A novel cell line, YST-1, was established from an epithelioid malignant schwannoma (EMS) that occurred in the upper arm of an 8-year-old girl. YST-1 cells were polygonal and stellate in shape, contained abundant free ribosomes, mitochondria, lysosomes and rough-surfaced endoplasmic reticulum, and grew stably with a population doubling time of 40 h. Immunohistochemically, vimentin, S100 protein and S100 protein beta subunit were positive in the cytoplasm. The xeno-transplanted tumor in nude mice was composed of cells with an epithelioid arrangement similar to the original tumor. The borders of the tumor cells were connected intimately without desmosomal junctions, and there were abundant organelles in the cytoplasm. YST-1 cells were considered to be of value for studying the nature and histogenesis of EMS., null, null, [Y Nagashima, Y Ohaki, Y Tanaka, K Sumino, T Funabiki, T Okuyama, S Watanabe, M Umeda, K Misugi], null, null, Cancer cell line, 1def3485-d42c-4154-b06f-589d02e1ad86, null, Schwannoma cell line, potentially mischaracterized in some cases as a sporadic MPNST cell line., No known disease, 10.1007/BF02899420, b3964ac1-90da-4af6-b6d8-0dbe343167c2, null, null, null, null, null, null, Virchows Archiv. B, Cell Pathology Including Molecular Pathology, [schwannoma], unknown, null, null, null, null, 2a2cb57f-86ca-42cf-aad2-930aae16339b, Asian, null, 617cb183-3377-4473-8ad8-2f6472bce1fa, YST-1, cellLine, RRID:CVCL_5192, Female, Homo sapiens, null, null, [YST1], null, null, schwannoma, [unknown], null, null, null, null]

[abstract, animalModelId, animalState, authors, catalogNumber, catalogNumberUrl, cellLineCategory, cellLineId, contaminatedMisidentified, description, disease, doi, donorId, generation, geneticBackground, institution, investigatorId, investigatorName, investigatorWebsite, journal, modelOfManifestation, mtaRequired, orcid, organ, originYear, populationDoublingTime, publicationId, race, resistance, resourceId, resourceName, resourceType, rrid, sex, species, strProfile, strainNomenclature, synonyms, tissue, transplantationType, tumorType, usageRestrictions, vendorId, vendorItemId, vendorName, website]
[null, null, null, null, GM11602, null, Transformed cell line, a76cad86-d2a2-402d-bb06-5ee13a8cdcec, null, Leukemia cell line derived from B-lymphocytes from an NF1 patient., Neurofibromatosis type 1, null, 49b6cb1f-7231-4208-a012-41c9bffff993, null, null, null, null, null, null, null, [acute lymphocytic leukemia], unknown, null, null, null, null, null, White, null, f354ec1a-0305-4e6c-897a-10e4fba10a28, GM11602, cellLine, RRID:CVCL_AA02, Female, Homo sapiens, null, null, null, null, null, acute lymphocytic leukemia, [unknown], 6c22c677-407b-463c-b53c-6dfa3c50e681, 56da4429-da43-44c8-862b-8a70955091df, Corielle Institute, https://www.coriell.org]
[null, null, null, null, GM11601, null, Transformed cell line, 58d36942-062c-4db1-91f9-ee8cda50f4fc, null, Leukemia cell line derived from B-lymphocytes from an NF1 patient., Neurofibromatosis type 1, null, 0460814f-4395-46bd-8acf-f1ded8afde9d, null, null, null, null, null, null, null, [acute lymphocytic leukemia], unknown, null, null, null, null, null, White, null, a2f62c57-a1b8-4867-9c91-04406f261cfa, GM11601, cellLine, RRID:CVCL_AA01, Male, Homo sapiens, null, null, null, null, null, acute lymphocytic leukemia, [unknown], 6c22c677-407b-463c-b53c-6dfa3c50e681, 495b8f81-36bc-4273-8e78-27ed87ef48c1, Corielle Institute, https://www.coriell.org]
[Skin fibroblast cell strains and tumour cell lines were established from 12 patients with various types of soft tissue neoplasms, and radiation survival curve parameters were measured in vitro. Soft tissue sarcoma cells were consistently more sensitive to X-irradiation than fibroblasts isolated from the same patient, and were also more sensitive as a group than cell lines derived from 34 other human tumours. There was a general correlation in radiosensitivity between fibroblasts and tumour cells derived from the same patient, indicating that some component of tumour cell sensitivity may relate to genetic factors in the host. Such genetic factors, however, do not explain all of the heterogeneity in tumour cell response. The response of soft tissue sarcoma in vivo may be dependent on complex radiomodifying factors other than inherent radiation sensitivity, thus making it difficult to predict clinical outcome by use of assays which use survival of irradiated tumour cell lines in vitro as an endpoint., null, null, [W K Dahlberg, J B Little, J A Fletcher, H D Suit, P Okunieff], null, null, Cancer cell line, 1139e45c-9645-449f-a22e-7497cbffb0b3, null, Sporadic MPNST tumor cell line from a non-NF1 patient., Cancer, 10.1080/09553009314550251, b50e7512-a686-4c50-9e95-6af9d06bf826, null, null, null, null, null, null, International Journal of Radiation Biology, [malignant peripheral nerve sheath tumor], unknown, null, null, null, null, 979d13af-cd42-4fcf-ad7f-bd52e4b28bcf, null, null, 9a7997c9-9399-47ed-b44b-5b717be89ba3, STS-26T, cellLine, RRID:CVCL_8917, Female, Homo sapiens, null, null, [STS26, STS26T], null, null, malignant peripheral nerve sheath tumor, [unknown], null, null, null, null]
[A novel cell line, YST-1, was established from an epithelioid malignant schwannoma (EMS) that occurred in the upper arm of an 8-year-old girl. YST-1 cells were polygonal and stellate in shape, contained abundant free ribosomes, mitochondria, lysosomes and rough-surfaced endoplasmic reticulum, and grew stably with a population doubling time of 40 h. Immunohistochemically, vimentin, S100 protein and S100 protein beta subunit were positive in the cytoplasm. The xeno-transplanted tumor in nude mice was composed of cells with an epithelioid arrangement similar to the original tumor. The borders of the tumor cells were connected intimately without desmosomal junctions, and there were abundant organelles in the cytoplasm. YST-1 cells were considered to be of value for studying the nature and histogenesis of EMS., null, null, [Y Nagashima, Y Ohaki, Y Tanaka, K Sumino, T Funabiki, T Okuyama, S Watanabe, M Umeda, K Misugi], null, null, Cancer cell line, 1def3485-d42c-4154-b06f-589d02e1ad86, null, Schwannoma cell line, potentially mischaracterized in some cases as a sporadic MPNST cell line., No known disease, 10.1007/BF02899420, b3964ac1-90da-4af6-b6d8-0dbe343167c2, null, null, null, null, null, null, Virchows Archiv. B, Cell Pathology Including Molecular Pathology, [schwannoma], unknown, null, null, null, null, 2a2cb57f-86ca-42cf-aad2-930aae16339b, Asian, null, 617cb183-3377-4473-8ad8-2f6472bce1fa, YST-1, cellLine, RRID:CVCL_5192, Female, Homo sapiens, null, null, [YST1], null, null, schwannoma, [unknown], null, null, null, null]

No results