Defective DNA repair and neurodegenerative disease

Rass, Ulrich, Ahel, Ivan and West, Stephen C (2007) Defective DNA repair and neurodegenerative disease. Cell, 130 (6). pp. 991-1004. ISSN 0092-8674

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Abstract

Defects in cellular DNA repair processes have been linked to genome instability, heritable cancers, and premature aging syndromes. Yet defects in some repair processes manifest themselves primarily in neuronal tissues. This review focuses on studies defining the molecular defects associated with several human neurological disorders, particularly ataxia with oculomotor apraxia 1 (AOA1) and spinocerebellar ataxia with axonal neuropathy 1 (SCAN1). A picture is emerging to suggest that brain cells, due to their nonproliferative nature, may be particularly prone to the progressive accumulation of unrepaired DNA lesions.

Item Type: Article
Keywords: DNA repair, neurodegenerative disease, DNA single-strand break repair, abortive DNA ligation, histidine triad, AMP-DNA, DNA de-adenylation, aprataxin, ataxia with oculomotor apraxia 1, tyrosyl-DNA phospho- diesterase 1, TDP1, covalent topoisomerase I-DNA adducts, pinocerebellar ataxia with axonal neuropathy 1
Schools and Departments: School of Life Sciences > Sussex Centre for Genome Damage and Stability
Research Centres and Groups: Genome Damage and Stability Centre
Subjects: Q Science > Q Science (General)
Depositing User: Ulrich Rass
Date Deposited: 26 Sep 2018 08:08
Last Modified: 26 Sep 2018 08:08
URI: http://srodev.sussex.ac.uk/id/eprint/79013
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