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Human Schwann Cell-Like: Cell line sNF94.3

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*Abstract

Cell Line Obtained From a Lung Tumor in a Patient With Neurofibromatosis-1Scientists at the University of Florida have developed a wide variety of monoclonal and polyclonal antibodies, cell lines, vectors and biological materials that can be used in other research projects. Anyone interested in accessing these materials via a license from the University of Florida should contact our office to make arrangements. NF1 Status: The MPNST culture sNF94.3 was derived from tumor material from a 43 year-old female NF1 patient who met diagnostic criteria. Although there was no family history, the patient had definite features of NF1 including a mild learning disability, scoliosis, café-au-lait spots, Lisch nodules, hundreds of dermal neurofibromas, a congenital plexiform in the ankle and foot, and a MPNST in the thigh. The originative tumor tissue for the sNF94.3 cell line was obtained from a lung metastasis diagnosed by histopathology as a MPNST. The portion of the tumor specimen used for tissue culture was independently characterized by immunohistopathology and confirmed as MPNST. The sNF94.3 tumor culture was established from numerous passages of primary tumor material in culture until they were a homogenous Schwann cell-like population and displayed a clonal morphology immunopositive for both the cytoplasmic Schwann cell markers S100 and p75. sNF94.3 has a normal karyotype, and the germline and somatic NF1 mutations remain unknown despite analysis of numerous exons. Interestingly, the LOH analysis was uninformative, showing homozygous markers across the NF1 gene. Thus, there may be a germline microdeletion in the region, which would be consistent with the patient's heavy dermal tumor burden and occurrence of the MPNST (De Raedt et al., 2003). These data predict this cell line should produce full-length neurofibromin protein. Western immunoblotting showed apparently full-length neurofibromin, confirmed with several anti-neurofibromin antibodies (Perrin et al., manuscript in press). Abnormalities in sNF94.3's neurofibromin remain unknown.

*Principal Investigation

Name: David Muir

Department:

Name: Margaret Wallace

Department:

其他

国家/地区

美国