Mitochondrial DNA damage and repair in neurodegenerative disorders

Jenq Lin Yang, Lior Weissman, Vilhelm A. Bohr, Mark P. Mattson

Research output: Contribution to journalArticle

Abstract

By producing ATP and regulating intracellular calcium levels, mitochondria are vital for the function and survival of neurons. Oxidative stress and damage to mitochondrial DNA during the aging process can impair mitochondrial energy metabolism and ion homeostasis in neurons, thereby rendering them vulnerable to degeneration. Mitochondrial abnormalities have been documented in all of the major neurodegenerative disorders-Alzheimer's, Parkinson's and Huntington's diseases, and amyotrophic lateral sclerosis. Mitochondrial DNA damage and dysfunction may be downstream of primary disease processes such as accumulation of pathogenic proteins. However, recent experimental evidence demonstrates that mitochondrial DNA damage responses play important roles in aging and in the pathogenesis of neurodegenerative diseases. Therapeutic interventions that target mitochondrial regulatory systems have been shown effective in cell culture and animal models, but their efficacy in humans remains to be established.

Original languageEnglish (US)
Pages (from-to)1110-1120
Number of pages11
JournalDNA Repair
Volume7
Issue number7
DOIs
StatePublished - Jul 1 2008
Externally publishedYes

Keywords

  • Alzheimer's disease
  • Apoptosis
  • Base excision repair
  • Parkinson's disease
  • Superoxide anion radical
  • Uracil DNA glycosylase

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology

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  • Cite this

    Yang, J. L., Weissman, L., Bohr, V. A., & Mattson, M. P. (2008). Mitochondrial DNA damage and repair in neurodegenerative disorders. DNA Repair, 7(7), 1110-1120. https://doi.org/10.1016/j.dnarep.2008.03.012