Ataxia-telangiectasia, cancer and the pathobiology of the ATM gene

被引：147

作者：

Meyn, MS

机构：

[1] Univ Toronto, Genet & Genomic Biol Program, Hosp Sick Children, Dept Paediat, Toronto, ON M5G 1X8, Canada

[2] Univ Toronto, Genet & Genomic Biol Program, Hosp Sick Children, Dept Mol & Med Genet, Toronto, ON M5G 1X8, Canada

来源：

CLINICAL GENETICS | 1999年 / 55卷 / 05期

关键词：

apoptosis; ataxia-telangiectasia; ATM; breast cancer; cell cycle checkpoints; leukemia;

D O I：

10.1034/j.1399-0004.1999.550501.x

中图分类号：

Q3 [遗传学];

学科分类号：

071007 ; 090102 ;

摘要：

The ability to maintain genomic integrity in the face of DNA damage is critical for survival. Biological organisms are not merely passive targets of DNA-damaging agents, but actively respond to DNA damage in a variety of ways, e.g. the SOS system in Escherichia coli(1). The means by which cells achieve this goal are complex and involve DNA repair, genetic recombination, alterations in the cell cycle, and programmed cell death. This article reviews recent developments in our understanding of ataxia-telangiectasia (A-T), a human disease in which these homeostatic processes have broken down, resulting: in neurologic degeneration, cancer, immunodeficiency, and mutagen sensitivity.

引用

页码：289 / 304

页数：16

共 183 条

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