Minimizing the damage: repair pathways keep mitochondrial DNA intact

Nat Rev Mol Cell Biol. 2012 Oct;13(10):659-71. doi: 10.1038/nrm3439. Epub 2012 Sep 20.


Mitochondrial DNA (mtDNA) faces the universal challenges of genome maintenance: the accurate replication, transmission and preservation of its integrity throughout the life of the organism. Although mtDNA was originally thought to lack DNA repair activity, four decades of research on mitochondria have revealed multiple mtDNA repair pathways, including base excision repair, single-strand break repair, mismatch repair and possibly homologous recombination. These mtDNA repair pathways are mediated by enzymes that are similar in activity to those operating in the nucleus, and in all cases identified so far in mammals, they are encoded by nuclear genes.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cell Nucleus / genetics
  • Cell Nucleus / metabolism
  • DNA Breaks*
  • DNA Ligases / metabolism
  • DNA Repair*
  • DNA, Mitochondrial / genetics
  • DNA, Mitochondrial / metabolism*
  • DNA-(Apurinic or Apyrimidinic Site) Lyase / metabolism
  • Humans
  • Mitochondria / genetics
  • Mitochondria / metabolism


  • DNA, Mitochondrial
  • DNA-(Apurinic or Apyrimidinic Site) Lyase
  • DNA Ligases