Oncogenes in radioresistant, noncancerous skin fibroblasts from a cancer-prone family.
Li-Fraumeni syndrome is manifested in a variety of neoplasms that are transmitted in a dominantly inherited pattern. The noncancerous skin fibroblasts of family members exhibit a unique characteristic of being resistant to the killing effect of ionizing radiation. A three- to eightfold elevation in expression of c-myc and an apparent activation of c-raf-1 gene have been observed in these noncancerous skin fibroblasts. These results may provide insight into the heritable defect underlying the familial predisposition to a variety of cancers.
D000818:Animals; D002471:Cell Transformation, Neoplastic / Q000528:radiation effects; D002478:Cells, Cultured; D005347:Fibroblasts / Q000528:radiation effects*; D006801:Humans; D051379:Mice; D008819:Mice, Nude; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics*; D009857:Oncogenes / Q000528:radiation effects*; D010375:Pedigree; D011836:Radiation Tolerance; D012867:Skin / Q000166:cytology / Q000528:radiation effects*; D013577:Syndrome