Mitochondrial DNA Repair in Mammals

Abstract

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

References

Anson R, Senturker S, Dizdaroglu M and Bohr V (1999) Measurement of oxidatively induced base lesions in liver from Wistar rats of different ages. Free Radical Biology and Medicine 27: 456–462.

Chen D, Cao G, Hastings T, et al. (2002) Age‐dependent decline of DNA repair activity for oxidative lesions in rat brain mitochondria. Journal of Neurochemistry 81: 1273–1284.

Coller H, Khrapko K, Bodyak N, et al. (2001) High frequency of homoplasmic mitochondrial DNA mutations in human tumors can be explained without selection. Nature Genetics 28: 147–150.

Dobson AW, Xu Y, Kelley MR, LeDoux SP and Wilson GL (2000) Enhanced mitochondrial DNA repair and cellular survival after oxidative stress by targeting the human 8‐oxoguanine glycosylase repair enzyme to mitochondria. Journal of Biological Chemistry 275: 37518–37523.

Longley MJ, Nguyen D, Kunkel TA and Copeland WC (2001) The fidelity of human DNA polymerase gamma with and without exonucleolytic proofreading and the p55 accessory subunit. Journal of Biological Chemistry 276: 38555–38562.

Penta J, Johnson F, Wachsman J and Copeland W (2001) Mitochondrial DNA in human malignancy. Mutation Research 488: 119–133.

Pinz KG and Bogenhagen DF (1998) Efficient repair of abasic sites in DNA by mitochondrial enzymes. Molecular and Cellular Biology 18: 1257–1265.

Pinz KS and Bogenhagen DF (2000) Characterization of a catalytically slow AP lyase activity in DNA polymerase and other family A DNA polymerases. Journal of Biological Chemistry 275: 12509–12514.

Pinz KG, Shibutani S and Bogenhagen DF (1995) Action of mitochondrial DNA polymerase gamma at sites of base loss or oxidative damage. Journal of Biological Chemistry 270: 9202–9206.

Ponamarev MV, Longley MJ, Nguyen D, Kunkel TA and Copeland WC (2002) Active site mutation in DNA polymerase gamma associated with progressive external ophthalmoplegia causes error‐prone DNA synthesis. Journal of Biological Chemistry 277: 15225–15228.

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 http://www.mitomap.org

APEX1 (APEX nuclease (multifunctional DNA repair enzyme) 1); LocusID: 328. LocusLink: http://www.ncbi.nlm.nih.gov/LocusLink/LocRpt.cgi?l=328

APEX1 (APEX nuclease (multifunctional DNA repair enzyme) 1); MIM number: 107748. OMIM: http://www.ncbi.nlm.nih.gov/htbin‐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. http://www.els.net [doi: 10.1038/npg.els.0005969]