Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerves and result in high rates of local recurrence and hematogenous metastasis. Half of MPNSTs occur in patients with neurofibromatosis type 1 (NF1), a common autosomal dominant multisystem disorder caused by mutations of the NF1 tumor suppressor gene located on chromosome 17q. Loss of function of the intact NF1 allele, due to loss of heterozygosity (LOH) and somatic mutations of the gene, is associated with dermal and plexiform neurofibroma formation. Approximately 10% of NF1 patients develop MPNST, of whom only 21% survive for five years after diagnosis.

Each vial contains ≥ 1X10⁶ viable cells.Cells are tested negative for infectious diseases by a Human Essential CLEAR panel by Charles River Animal Diagnostic Services.Cells are verified to be of human origin and negative for inter-species contamination from mouse, rat, Chinese hamster, Golden Syrian hamster, and Non-human Primate (NHP) as assessed by a Contamination Clear panel by Charles River Animal Diagnostic Services Cells are negative for mycoplasma contamination.Each lot of cells are genotyped by STR analysis to verify the unique identity of the cell line.

Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerves and result in high rates of local recurrence and hematogenous metastasis. Half of MPNSTs occur in patients with neurofibromatosis type 1 (NF1), a common autosomal dominant multisystem disorder caused by mutations of the NF1 tumor suppressor gene located on chromosome 17q. Loss of function of the intact NF1 allele, due to loss of heterozygosity (LOH) and somatic mutations of the gene, is associated with dermal and plexiform neurofibroma formation. Approximately 10% of NF1 patients develop MPNST, of whom only 21% survive for five years after diagnosis. Source S462 is an established human MPNST cell line from a clinically and genetically well-characterized NF1 patient, with a nonsense germline mutation in exon 37 of the NF1 gene, LOH of the NF1 gene, LOH of the p53 gene, and a homozygous missense mutation in exon 4 of the p53gene.As a verified MPNST cell line, S462 is a valuable tool for the further investigation of the biology and pathogenesis of this malignancy as well as for in vitro pharmacologic studies essential for the development of new therapies.

S462 Human Peripheral Nerve Sheath Tumor Cell Line is a varified MPNST cell line and a valuable tool for the further investigation of the biology and pathogenesis of this malignancy.

Cells should be stored in liquid nitrogen. The cells can be cultured for at least 10 passages after initial thawing without significantly affecting the cell marker expression and functionality.

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