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Radiation hypersensitivity and radioresistant DNA synthesis in ataxia-telangiectasia

Conference · · Trans. Am. Nucl. Soc.; (United States)
OSTI ID:5177199
Patients with the autosomal recessive genetic disease, ataxia-telangiectasia (A-T), are cancer-prone and hypersensitive to the killing effects of ionizing radiation. In an attempt to isolate the gene(s) responsible for the hypersensitivity of A-T cells, they were transfected with normal human DNA in cosmid vectors containing a rescuable marker (G-418 resistance), and revertants to normal sensitivity were isolated and characterized. The failure of radioresistant revertants to demonstrate a reversion of the phenotype, radioresistant DNA synthesis, shows that this feature is dependent on a gene separate from the one conferring resistance to cell killing. Cells from every A-T patient thus far examined demonstrate both hypersensitivity, in terms of radiation-induced cell killing, and radioresistant DNA synthesis. The results reported here, however, show that the former is not a result of the latter, as previously proposed. Moreover, the fact that these two characteristics can be uncoupled obscures the role(s) that either of them plays in the etiology of the disease, or in the development in its other features, including cancer-proneness.
Research Organization:
Univ. of California, San Francisco
OSTI ID:
5177199
Report Number(s):
CONF-851115-
Conference Information:
Journal Name: Trans. Am. Nucl. Soc.; (United States) Journal Volume: 50
Country of Publication:
United States
Language:
English