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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
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Cells 2019, 8(4), 379; https://doi.org/10.3390/cells8040379
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)
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Abstract

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

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