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Int. J. Mol. Sci. 2017, 18(8), 1821; doi:10.3390/ijms18081821

Principal Aspects Regarding the Maintenance of Mammalian Mitochondrial Genome Integrity

1
Department of Basic Medical Sciences, Laboratory of Histology & Embryology, School of Medicine, National and Kapodistrian University of Athens, 75 MikrasAsias Avenue, Goudi, Athens 11527, Greece
2
Department of Pharmacology, School of Medicine, National and Kapodistrian University of Athens, 75 MikrasAsias Avenue, Goudi, Athens 11527, Greece
*
Author to whom correspondence should be addressed.
Received: 10 July 2017 / Revised: 11 August 2017 / Accepted: 14 August 2017 / Published: 22 August 2017
(This article belongs to the Special Issue Mechanisms Leading to Genomic Instability)
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Abstract

Mitochondria have emerged as key players regarding cellular homeostasis not only due to their contribution regarding energy production through oxidative phosphorylation, but also due to their involvement in signaling, ion regulation, and programmed cell death. Indeed, current knowledge supports the notion that mitochondrial dysfunction is a hallmark in the pathogenesis of various diseases. Mitochondrial biogenesis and function require the coordinated action of two genomes: nuclear and mitochondrial. Unfortunately, both intrinsic and environmental genotoxic insults constantly threaten the integrity of nuclear as well as mitochondrial DNA. Despite the extensive research that has been made regarding nuclear genome instability, the importance of mitochondrial genome integrity has only recently begun to be elucidated. The specific architecture and repair mechanisms of mitochondrial DNA, as well as the dynamic behavior that mitochondria exert regarding fusion, fission, and autophagy participate in mitochondrial genome stability, and therefore, cell homeostasis. View Full-Text
Keywords: genomic instability; mitochondrial genome; nucleoids; mitochondrial DNA repair mechanisms; heteroplasmy; fusion; fission; mitophagy genomic instability; mitochondrial genome; nucleoids; mitochondrial DNA repair mechanisms; heteroplasmy; fusion; fission; mitophagy
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Vasileiou, P.V.S.; Mourouzis, I.; Pantos, C. Principal Aspects Regarding the Maintenance of Mammalian Mitochondrial Genome Integrity. Int. J. Mol. Sci. 2017, 18, 1821.

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