Enhanced viral transformation of skin fibroblasts from neurofibromatosis patients.
Enhanced viral transformation of cultured skin fibroblasts (SF) from patients with neurofibromatosis (NF) was observed, compared with cultures established from normal, age-matched controls. Cultures of skin fibroblasts from persons with and without clinical NF in families in which the disorder had been diagnosed were examined for transformability by Kirsten murine sarcoma virus. The viral transformation results were compared with those obtained with SF cultures initiated from controls in families without history of any disorder with an hereditary component, or cancer. The data show that 63 percent of cultures from patients with clinical NF were transformed, compared with 7 percent of control cultures (P = less than 0.0054). Cultures of skin fibroblasts from persons without the classical features of NF, but in families in which the disorder had been recognized, also exhibited a relatively high transformation rate, since 75 percent were transformed. Neurofibromatosis can be included among other hereditary disorders in which enhanced transformability of cultures of SF by an oncogenic virus may be demonstrated.
D000328:Adult; D000368:Aged; D002472:Cell Transformation, Viral; D002478:Cells, Cultured; D005260:Female; D005347:Fibroblasts / Q000166:cytology; D006801:Humans; D007223:Infant; D007708:Kirsten murine sarcoma virus / Q000502:physiology; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000235:genetics / Q000473:pathology*; D012867:Skin / Q000166:cytology*