methylnitronitrosoguanidine and Neuromuscular-Diseases

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.

Topics: Cell Survival; Fibroblasts; Humans; Huntington Disease; Methylnitronitrosoguanidine; Muscular Dystrophies; Neuromuscular Diseases; Reference Values; Syndrome; Tritium; X-Rays