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Defective DNA repair and neurodegenerative disease
journal contribution
posted on 2023-06-09, 15:13 authored by Ulrich RassUlrich Rass, Ivan Ahel, Stephen C WestDefects 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.
History
Publication status
- Published
Journal
CellISSN
0092-8674Publisher
ElsevierExternal DOI
Issue
6Volume
130Page range
991-1004Department affiliated with
- Sussex Centre for Genome Damage Stability Publications
Research groups affiliated with
- Genome Damage and Stability Centre Publications
Full text available
- No
Peer reviewed?
- Yes
Legacy Posted Date
2018-09-26Usage metrics
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