title: Genetic effects on the longevity of cultured human fibroblasts. III. Correlations with altered glucose-6-phosphate dehydrogenase.
abstract: The level of heat-labile glucose-6-phosphate dehydrogenase (G6PD) has been measured in skin fibroblast cultures from premature ageing or DNA repair deficient genetic syndromes. The short in vitro longevity of Werner's syndrome, progeria, Cockayne's syndrome, ataxia telangiectasia, Fanconi's anaemia, and Bloom's syndrome cultures was correlated with the appearance of a significant fraction of heat-labile enzyme. Long-lived control cultures contain a low level of altered enzyme until they become senescent. The evidence that heat-labile G6PD molecules are derived from errors in synthesis, or from other causes, is critically assessed. It is shown that normal cells grown in medium containing the antibiotic, paromomycin, which is known to reduce the fidelity of ribosomal translation, produce a significant fraction of altered G6PD.
mesh_terms: D000368:Aged; D001260:Ataxia Telangiectasia / Q000201:enzymology / Q000235:genetics; D002470:Cell Survival; D002478:Cells, Cultured; D003057:Cockayne Syndrome / Q000201:enzymology / Q000235:genetics; D004260:DNA Repair; D005199:Fanconi Anemia / Q000201:enzymology / Q000235:genetics; D005347:Fibroblasts / Q000187:drug effects / Q000201:enzymology / Q000502:physiology*; D005954:Glucosephosphate Dehydrogenase / Q000032:analysis* / Q000096:biosynthesis / Q000378:metabolism; D006801:Humans; D010303:Paromomycin / Q000494:pharmacology; D011371:Progeria / Q000201:enzymology / Q000235:genetics; D014176:Protein Biosynthesis; D012867:Skin / Q000473:pathology / Q000503:physiopathology; D014898:Werner Syndrome / Q000201:enzymology / Q000235:genetics
chemical_list: D010303:Paromomycin; D005954:Glucosephosphate Dehydrogenase