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Mitochondrial DNA: Distribution, Mutations, and Elimination

Hubei Key Laboratory of Cell Homeostasis, College of Life Sciences, Wuhan University, Wuhan 430072, China
Author to whom correspondence should be addressed.
Cells 2019, 8(4), 379;
Received: 1 April 2019 / Revised: 17 April 2019 / Accepted: 20 April 2019 / Published: 25 April 2019
(This article belongs to the Special Issue Mitochondrial Genetics)
PDF [1043 KB, uploaded 26 April 2019]


Mitochondrion harbors its own DNA (mtDNA), which encodes many critical proteins for the assembly and activity of mitochondrial respiratory complexes. mtDNA is packed by many proteins to form a nucleoid that uniformly distributes within the mitochondrial matrix, which is essential for mitochondrial functions. Defects or mutations of mtDNA result in a range of diseases. Damaged mtDNA could be eliminated by mitophagy, and all paternal mtDNA are degraded by endonuclease G or mitophagy during fertilization. In this review, we describe the role and mechanism of mtDNA distribution and elimination. In particular, we focus on the regulation of paternal mtDNA elimination in the process of fertilization. View Full-Text
Keywords: mitochondria; mtDNA; mitophagy; mtDNA distribution; mitochondrial dynamics mitochondria; mtDNA; mitophagy; mtDNA distribution; mitochondrial dynamics

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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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Yan, C.; Duanmu, X.; Zeng, L.; Liu, B.; Song, Z. Mitochondrial DNA: Distribution, Mutations, and Elimination. Cells 2019, 8, 379.

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