Mitochondrial DNA Repair in Mammals


In the past it was assumed that damaged mitochondrial DNA molecules in mammalian cells were not repaired, but it is now known that editing of polymerase errors and base excision repair are functional in mammalian mitochondria.

Keywords: mitochondrial genome; mitochondrial disorders; oxidative stress; base excision repair; DNA acid replication fidelity


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Further Reading

Bogenhagen DF (1999) Repair of mtDNA in vertebrates. American Journal of Human Genetics 64: 1276–1281.

Chinnery P and Turnbull D (2001) Epidemiology and treatment of mitochondrial disorders. American Journal of Medical Genetics 106: 94–101.

LeDoux S, Driggers W, Hollensworth B and Wilson G (1999) Repair of alkylation and oxidative damage in mitochondrial DNA. Mutation Research 434: 149–159.

Lindahl T (1993) Instability and decay of the primary structure of DNA. Nature 362: 709–715.

Nakabeppu Y (2001) Regulation of intracellular localization of human MTH1, OGG1 and MYH proteins for repair of oxidative DNA damage. Progress in Nucleic Acids Research and Molecular Biology 68: 75–94.

Raha S and Robinson B (2000) Mitochondria, oxygen free radicals, disease and aging. Trends in Biochemical Science 25: 502–508.

Singh G, Sharkey SM and Moorehead R (1992) Mitochondrial DNA damage by anticancer agents. Pharmacological Therapy 54: 217–230.

Wallace DC (1992) Mitochondrial genetics: a paradigm for aging and degenerative diseases? Science 256: 628–632.

Web Links

Mitomap. A human mitochondrial genome database

APEX1 (APEX nuclease (multifunctional DNA repair enzyme) 1); LocusID: 328. LocusLink:

APEX1 (APEX nuclease (multifunctional DNA repair enzyme) 1); MIM number: 107748. OMIM:‐post/Omim/dispmim?107748

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How to Cite close
Bogenhagen, Daniel F(Jan 2006) Mitochondrial DNA Repair in Mammals. In: eLS. John Wiley & Sons Ltd, Chichester. [doi: 10.1038/npg.els.0005969]