Studies of cellular radiosensitivity in hereditary disorders of nervous system and muscle.
Skin fibroblasts from patients with familial dysautonomia, Duchenne muscular dystrophy and Charcot-Marie-Tooth disease show normal sensitivity to ionising radiation, as measured by post-irradiation clonal growth. Previous reports of cellular hypersensitivity to ionising radiation and other DNA-damaging agents in familial dysautonomia and Duchenne muscular dystrophy have not been confirmed.
D000293:Adolescent; D000328:Adult; D002607:Charcot-Marie-Tooth Disease / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004402:Dysautonomia, Familial / Q000503:physiopathology*; D005260:Female; D005347:Fibroblasts / Q000528:radiation effects*; D006801:Humans; D008297:Male; D009133:Muscular Atrophy / Q000503:physiopathology*; D009136:Muscular Dystrophies / Q000503:physiopathology*