PMID: 8958160Jan 1, 1997Paper

Hypersensitivity of ataxia-telangiectasia fibroblasts to a nitric oxide donor

Free Radical Biology & Medicine
M H GreenC F Arlett

Abstract

Ataxia-telangiectasia (A-T) is a human autosomal recessive disease characterised by immunodeficiency, extreme sensitivity to ionising radiation and progressive cerebellar ataxia. The defective gene has recently been cloned and is a member of the phosphatidylinositol 3-kinase family. We have investigated the possibility that the neurodegeneration in A-T might be induced by an endogenously formed mutagen causing radiation-like damage. Nitric oxide is known to be formed in the cerebellum and we present evidence that A-T fibroblasts are hypersensitive to killing by the nitric oxide donor S-nitrosoglutathione (GSNO), as are fibroblasts from a radiosensitive individual without ataxia. Killing was determined as loss of colony forming ability. GSNO induces dose-dependent DNA strand breakage, but to no greater extent in A-T fibroblasts. Breakdown of GSNO to nitrite and nitrate appears to occur to the same extent in both normal and A-T fibroblasts. Cell killing by GSNO appears to be associated in both types of cell with formation of nitrite, rather than nitrate, as the ultimate oxidation product of nitric oxide.

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Citations

Mar 18, 2006·Apoptosis : an International Journal on Programmed Cell Death·V D Nair
Jul 24, 1998·Mutation Research·S ThomasM H Green
Oct 31, 2000·Radiotherapy and Oncology : Journal of the European Society for Therapeutic Radiology and Oncology·P B RogersC F Arlett
Aug 5, 2000·Free Radical Biology & Medicine·R E ShackelfordR S Paules
Sep 25, 2001·Trends in Cell Biology·A K Pearce, T C Humphrey
May 7, 1999·Environmental Health Perspectives·R E ShackelfordR S Paules
Jul 16, 2010·Geriatrics & Gerontology International·Yuri Miura, Tamao Endo
Mar 13, 2003·Redox Report : Communications in Free Radical Research·Dianne J Watters
Jan 17, 2015·The International Journal of Biochemistry & Cell Biology·Ji Hoon YuHyeyoung Kim
Jun 15, 2011·DNA Repair·Michaela SemlitschErnst Malle
Jan 11, 2018·Science Signaling·Ji-Hoon LeeTanya T Paull

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