The Response of Ataxia Telangiectasia Cells to Bleomycin

Overview

Journal Nucleic Acids Res

Publisher Oxford University Press

Specialty Biochemistry

Date 1979 Jan 1

PMID 88042

Citations 7

Authors

A R Lehmann

S Stevens

Affiliations

Soon will be listed here.

Abstract

The autosomal recessive disorder, ataxia telangiectasia (AT) is characterised by cellular sensitivity to ionizing radiation. The molecular basis of this radiosensitivity is the subject of controversy. We report here that cultured fibroblasts from AT patients are also sensitive to the lethal effects of bleomycin. As with ionizing radiation, no defect has been observed in the overall rejoining of single or double-strand breaks produced by bleomycin. Since, however, only apyrimidinic (and to a lesser extent apurinic) sites and strand breaks are known to be produced by bleomycin, we tentatively suggest that AT cells are unable to rejoin a very small fraction of the total strand breaks. We attribute our inability to detect such unrejoined strand breaks to the relative insensitivity of the sucrose gradient procedures normally used to detect strand breaks.

Citing Articles

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Genetic and biochemical studies with ataxia telangiectasia. A review.

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PMID: 10819014 DOI: 10.1007/BF00278846.

Neurological and cytogenetic study in early-onset ataxia-telangiectasia patients.

Leuzzi V, Elli R, Antonelli A, Chessa L, Cardona F, Marcucci L Eur J Pediatr. 1993; 152(7):609-12.

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Ataxia-telangiectasia: an inherited disorder of ionizing-radiation sensitivity in man. Progress in the elucidation of the underlying biochemical defect.

McKinnon P Hum Genet. 1987; 75(3):197-208.

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