Complementation of the defects of DNA synthesis in irradiated and unirradiated ataxia-telangiectasia cells.
AUTOR(ES)
Murnane, J P
RESUMO
Mutant subtypes in the human genetic disease ataxia-telangiectasia (A-T) were classified by means of an assay system that monitors complementation of defects in DNA synthesis. Anomalies in DNA synthesis have been observed previously in A-T cells, both in their failure to inhibit DNA synthesis immediately after exposure to ionizing radiation and in their prolonged S phase. Polyethylene glycol-mediated cell fusion and autoradiography were combined with selective identification of different A-T cell populations by fluorescent colored microspheres to determine complementation capabilities of various A-T cell combinations. Five complementation groups were identified by both a 30-40% increase in the rate of DNA synthesis in unirradiated heterokaryons and the appearance of normal inhibition of DNA synthesis after x-irradiation of heterokaryons. The correlation observed between these phenomena suggests that the defects in A-T cells involve problems in initiation of DNA synthesis.
ACESSO AO ARTIGO
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=346101Documentos Relacionados
- Reduced DNA topoisomerase II activity in ataxia-telangiectasia cells.
- Pleiotropic defects in ataxia-telangiectasia protein-deficient mice
- Purification and DNA binding properties of the ataxia-telangiectasia gene product ATM
- Pathological findings in three cases of ataxia-telangiectasia
- Genetic heterogeneity in ataxia-telangiectasia studied by cell fusion.