Evaluations of wasted mouse fibroblasts and SV-40 transformed human fibroblasts as models of ataxia telangiectasia in vitro.
Fibroblast cultures from wasted mice have been derived and the responses of these cultures to bleomycin treatment or gamma-irradiation have been examined. No differences were observed between wasted fibroblasts and littermate controls in the post-treatment inhibition of DNA replication. In contrast, a human SV-40 transformed ataxia telangiectasia fibroblast line mimicked the abnormal response of primary ataxia telangiectasia fibroblasts to gamma-rays or bleomycin and thus appears to be a useful in vitro model of ataxia telangiectasia.
D000818:Animals; D001260:Ataxia Telangiectasia / Q000473:pathology*; D001761:Bleomycin / Q000494:pharmacology; D002460:Cell Line; D002470:Cell Survival; D002472:Cell Transformation, Viral; D004261:DNA Replication / Q000187:drug effects / Q000528:radiation effects; D004195:Disease Models, Animal; D005720:Gamma Rays; D006801:Humans; D051379:Mice; D008817:Mice, Mutant Strains / Q000502:physiology*; D013539:Simian virus 40
D001761:Bleomycin