title: Cellular hypersensitivity to neocarzinostatin in ataxia-telangiectasia skin fibroblasts.
abstract: Cellular sensitivity of human skin fibroblast strains from three healthy donors, eight ataxia-telangiectasia (A-T) patients belonging to six sibships, and two A-T heterozygotes to the lethal action of the antitumor antibiotic neocarzinostatin was tested, using colony-forming ability as the criterion for survival. All the A-T strains were significantly more sensitive to killing by neocarzinostatin than were the control strains. The average D0 for the A-T strains following neocarzinostatin treatment was 14.6 ng/ml, as compared to 37.9 ng/ml for the normal strains. The two A-T heterozygous strains showed intermediate sensitivity with an average D0 of 26.9 ng/ml. Neocarzinostatin sensitivity of A-T cells could therefore serve as a convenient aid for the laboratory diagnosis of A-T. Since A-T cells are also known to be hypersensitive to ionizing radiation and bleomycin, it would appear that they are primarily hypersensitive to DNA-breaking agents.
mesh_terms: D000293:Adolescent; D000328:Adult; D000903:Antibiotics, Antineoplastic / Q000494:pharmacology*; D001260:Ataxia Telangiectasia / Q000503:physiopathology*; D002449:Cell Aggregation / Q000187:drug effects; D002460:Cell Line; D002470:Cell Survival / Q000187:drug effects; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D005260:Female; D005347:Fibroblasts / Q000502:physiology; D006801:Humans; D007700:Kinetics; D008297:Male; D008875:Middle Aged; D012867:Skin / Q000503:physiopathology*; D009353:Zinostatin / Q000494:pharmacology*
chemical_list: D000903:Antibiotics, Antineoplastic; D009353:Zinostatin