Normal repair of ultraviolet-induced DNA damage in a hypersensitive strain of fibroblasts from a patient with Gardner's syndrome.
Gardner's syndrome is an autosomal dominant disorder that predisposes to cancer of the large intestine and to other tumors. We have previously demonstrated that fibroblasts from a patient with this disease are hypersensitive to the cytotoxic effects of ultraviolet light. In this report we have measured several parameters of the repair of ultraviolet light-induced DNA damage in an attempt to identify a defect responsible for the hypersensitivity. We have found the excision rate of pyrimidine dimers, the host cell reactivation of UV-irradiated herpes simplex virus, the induction and rejoining of DNA single strand breaks and the response of semi-conservative DNA replication to UV-irradiation to be in all cases indistinguishable from such phenomena in a variety of normal cells.
D002478:Cells, Cultured; D002843:Chromatin / Q000528:radiation effects; D004247:DNA / Q000528:radiation effects; D004260:DNA Repair; D004261:DNA Replication / Q000528:radiation effects; D005736:Gardner Syndrome / Q000235:genetics*; D006801:Humans; D011740:Pyrimidine Dimers / Q000378:metabolism; D014466:Ultraviolet Rays; D014779:Virus Replication / Q000528:radiation effects
D002843:Chromatin; D011740:Pyrimidine Dimers; D004247:DNA