title: Minimizing the damage: repair pathways keep mitochondrial DNA intact.
abstract: 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.
authors.affiliation: MRC-Mitochondrial Biology Unit, Wellcome Trust/MRC Building, Cambridge, UK. lk303@mrc-mbu.cam.ac.uk
authors.forename: Lawrence
authors.initials: L
authors.lastname: Kazak
authors.forename: Aurelio
authors.initials: A
authors.lastname: Reyes
authors.forename: Ian J
authors.initials: IJ
authors.lastname: Holt
mesh_terms: D000818:Animals; D002467:Cell Nucleus / Q000235:genetics / Q000378:metabolism; D053960:DNA Breaks; D011088:DNA Ligases / Q000378:metabolism; D004260:DNA Repair; D004272:DNA, Mitochondrial / Q000235:genetics / Q000378:metabolism*; D043603:DNA-(Apurinic or Apyrimidinic Site) Lyase / Q000378:metabolism; D006801:Humans; D008928:Mitochondria / Q000235:genetics / Q000378:metabolism
chemical_list: D004272:DNA, Mitochondrial; D043603:DNA-(Apurinic or Apyrimidinic Site) Lyase; D011088:DNA Ligases