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Senataxin, defective in ataxia oculomotor apraxia type 2, is involved in the defense against oxidative DNA damage


Suraweera, A and Becherel, OJ and Chen, P and Rundle, N and Woods, R and Nakamura, J and Gatei, M and Criscuolo, C and Filla, A and Chessa, L and Fusser, M and Epe, B and Guven, N and Lavin, MF, Senataxin, defective in ataxia oculomotor apraxia type 2, is involved in the defense against oxidative DNA damage, The Journal of Cell Biology, 177, (6) pp. 969-979. ISSN 1540-8140 (2007) [Refereed Article]

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Copyright 2007 Rockefeller University Press

DOI: doi:10.1083/jcb.200701042


A defective response to DNA damage is observed in several human autosomal recessive ataxias with oculomotor apraxia, including ataxia-telangiectasia. We report that senataxin, defective in ataxia oculomotor apraxia (AOA) type 2, is a nuclear protein involved in the DNA damage response. AOA2 cells are sensitive to H2O2, camptothecin, and mitomycin C, but not to ionizing radiation, and sensitivity was rescued with full-length SETX cDNA. AOA2 cells exhibited constitutive oxidative DNA damage and enhanced chromosomal instability in response to H2O2. Rejoining of H2O2-induced DNA double-strand breaks (DSBs) was significantly reduced in AOA2 cells compared to controls, and there was no evidence for a defect in DNA single-strand break repair. This defect in DSB repair was corrected by full-length SETX cDNA. These results provide evidence that an additional member of the autosomal recessive AOA is also characterized by a defective response to DNA damage, which may contribute to the neurodegeneration seen in this syndrome.

Item Details

Item Type:Refereed Article
Keywords:senataxin, ataxia oculomotor apraxia 2, DNA damage response
Research Division:Biomedical and Clinical Sciences
Research Group:Medical biochemistry and metabolomics
Research Field:Medical biochemistry - proteins and peptides (incl. medical proteomics)
Objective Division:Expanding Knowledge
Objective Group:Expanding knowledge
Objective Field:Expanding knowledge in the health sciences
UTAS Author:Guven, N (Dr Nuri Guven)
ID Code:79278
Year Published:2007
Web of Science® Times Cited:129
Deposited By:Pharmacy
Deposited On:2012-08-29
Last Modified:2013-03-18

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