Mitochondrial DNA repair pathways

Mutat Res. 1999 Jul 30;434(3):137-48. doi: 10.1016/s0921-8777(99)00025-7.


DNA repair mechanisms are fairly well characterized for nuclear DNA while knowledge regarding the repair mechanisms operable in mitochondria is limited. Several lines of evidence suggest that mitochondria contain DNA repair mechanisms. DNA lesions are removed from mtDNA in cells exposed to various chemicals. Protein activities that process damaged DNA have been detected in mitochondria. As will be discussed, there is evidence for base excision repair (BER), direct damage reversal, mismatch repair, and recombinational repair mechanisms in mitochondria, while nucleotide excision repair (NER), as we know it from nuclear repair, is not present.

Publication types

  • Review

MeSH terms

  • Aging / genetics
  • Alkylation
  • Animals
  • Cell Nucleus / metabolism
  • DNA Repair*
  • DNA, Mitochondrial*
  • Models, Biological
  • Mutation
  • Oxidative Stress
  • Rats
  • Recombination, Genetic
  • Uracil / metabolism


  • DNA, Mitochondrial
  • Uracil