IMR Press / FBL / Volume 21 / Issue 1 / DOI: 10.2741/4375

Frontiers in Bioscience-Landmark (FBL) is published by IMR Press from Volume 26 Issue 5 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.


Mitochondrial DNA damage induced autophagy, cell death, and disease

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1 Department of Pharmacology Chemical Biology, University of Pittsburgh, 15213-1863, USA
2 School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27516, USA
3 Nicholas School of the Environment, Duke University, Durham, NC 27708-0328, USA
Academic Editor:Robert William Gilkerson
Front. Biosci. (Landmark Ed) 2016, 21(1), 42–54;
Published: 1 January 2016
(This article belongs to the Special Issue Mitochondrial bioenergetics in human health)

Mammalian mitochondria contain multiple small genomes. While these organelles have efficient base excision removal of oxidative DNA lesions and alkylation damage, many DNA repair systems that work on nuclear DNA damage are not active in mitochondria. What is the fate of DNA damage in the mitochondria that cannot be repaired or that overwhelms the repair system? Some forms of mitochondrial DNA damage can apparently trigger mitochondrial DNA destruction, either via direct degradation or through specific forms of autophagy, such as mitophagy. However, accumulation of certain types of mitochondrial damage, in the absence of DNA ligase III (Lig3) or exonuclease G (EXOG), enzymes required for repair, can directly trigger cell death. This review examines the cellular effects of persistent damage to mitochondrial genomes and discusses the very different cell fates that occur in response to different kinds of damage.

Mitochondrial DNA
DNA repair
Human Disease
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