Biochemistry (Moscow)

, Volume 83, Issue 3, pp 233–249 | Cite as

Mechanisms of Mitochondrial DNA Repair in Mammals

  • L. A. ZinovkinaEmail author


Accumulation of mutations in mitochondrial DNA leads to the development of severe, currently untreatable diseases. The contribution of these mutations to aging and progress of neurodegenerative diseases is actively studied. Elucidation of DNA repair mechanisms in mitochondria is necessary for both developing approaches to the therapy of diseases caused by mitochondrial mutations and understanding specific features of mitochondrial genome functioning. Mitochondrial DNA repair systems have become a subject of extensive studies only in the last decade due to development of molecular biology methods. DNA repair systems of mammalian mitochondria appear to be more diverse and effective than it had been thought earlier. Even now, one may speak about the existence of mitochondrial mechanisms for the repair of single–and double–stranded DNA lesions. Homologous recombination also takes place in mammalian mitochondria, although its functional significance and molecular mechanisms remain obscure. In this review, I describe DNA repair systems in mammalian mitochondria, such as base excision repair (BER) and microhomology–mediated end joining (MMEJ) and discuss a possibility of existence of mitochondrial DNA repair mechanisms otherwise typical for the nuclear DNA, e.g., nucleotide excision repair (NER), mismatch repair (MMR), homologous recombination, and classical non–homologous end joining (NHEJ). I also present data on the mechanisms for coordination of the nuclear and mitochondrial DNA repair systems that have been actively studied recently.


mitochondria mitochondrial DNA mutations DNA repair BER MMEJ homologous recombination PARP1 





base excision repair


Cockayne syndrome protein A


Cockayne syndrome protein B


electron transport chain


exo/endonuclease G


flap structure–specific endonuclease 1


long patch base excision repair


microhomology–mediated end joining


mismatch repair


mitochondrial DNA


nucleotide excision repair


non–homologous end joining








poly(ADP–ribose) polymerase


mitochondrial RNA polymerase


ribosomal DNA


reactive oxygen species


short patch base excision repair


single–strand binding protein


tyrosyl–DNA phosphodiesterase 1


transcriptional factor A of mitochondria


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© Pleiades Publishing, Ltd. 2018

Authors and Affiliations

  1. 1.Lomonosov Moscow State UniversityFaculty of Bioengineering and BioinformaticsMoscowRussia

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