title: Radiation sensitivity of fibroblast strains from patients with Usher's syndrome, Duchenne muscular dystrophy, and Huntington's disease.
abstract: The colony-forming ability of 10 normal human fibroblast cell strains and of 10 strains representing 3 degenerative diseases of either nerve or muscle cells was determined after exposure of the cells to X-rays or beta-particles from tritiated water. Both methods of irradiation yielded similar comparative results. The fibroblast strains from the 5 Usher's syndrome patients and from 1 of the 2 Huntington's disease patients were hypersensitive to radiation, while those from the 3 Duchenne muscular dystrophy patients and the second Huntington's disease patient had normal sensitivity to radiation. These results indicate both disease-specific and strain-specific differences in the survival of fibroblasts after exposure to ionizing radiation.
mesh_terms: D002470:Cell Survival / Q000187:drug effects / Q000528:radiation effects; D005347:Fibroblasts / Q000166:cytology / Q000187:drug effects / Q000528:radiation effects; D006801:Humans; D006816:Huntington Disease / Q000235:genetics* / Q000473:pathology; D008769:Methylnitronitrosoguanidine / Q000494:pharmacology; D009136:Muscular Dystrophies / Q000235:genetics* / Q000473:pathology; D009468:Neuromuscular Diseases / Q000235:genetics* / Q000473:pathology; D012016:Reference Values; D013577:Syndrome; D014316:Tritium; D014965:X-Rays
chemical_list: D014316:Tritium; D008769:Methylnitronitrosoguanidine