Defective DNA repair and neurodegenerative disease.
review · Level V
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- Record sourced from PubMed, PMID 17889645.
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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.
Medical subject headings
- DNA Breaks, Single-Stranded
- DNA Repair
- DNA-Binding Proteins
- Genomic Instability
- Neurodegenerative Diseases
- Neurons
- Nuclear Proteins
- Phosphoric Diester Hydrolases