title: Repair of potentially lethal X-ray damage in fibroblasts derived from patients with hereditary and D-deletion retinoblastoma.
abstract: We examined X-ray induced potentially lethal damage repair (PLDR) in density inhibited plateau phase cultures of six fibroblast strains derived from patients with hereditary retinoblastoma and two patients with D-deletion retinoblastoma and compared them to three normal controls. PLD was measured in hereditary retinoblastoma (7 Gy exposure) and normal cells (7 and 9 Gy exposure) after 24 h repair time. PLD survival curves were performed at 2-9 Gy on six retinoblastoma and three normal control cell strains. Thus, PLDR was compared at equitoxic survival levels as well as after exposure to equal doses of radiation. Some retinoblastoma strains showed normal PLDR whereas others were possibly deficient. Implications of PLDR for susceptibility to radiation-induced and spontaneous tumours in hereditary retinoblastoma patients are discussed.
mesh_terms: D000293:Adolescent; D000328:Adult; D002460:Cell Line; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002901:Chromosomes, Human, 13-15; D004260:DNA Repair; D004307:Dose-Response Relationship, Radiation; D005134:Eye Neoplasms / Q000235:genetics*; D005260:Female; D005347:Fibroblasts / Q000528:radiation effects; D006801:Humans; D066298:In Vitro Techniques; D008297:Male; D011831:Radiation Genetics; D012175:Retinoblastoma / Q000235:genetics*; D013997:Time Factors