Mitochondrial DNA: Distribution, Mutations, and Elimination

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 f...

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Main Authors: Chaojun Yan, Xiaoying Duanmu, Ling Zeng, Bing Liu, Zhiyin Song
Format: Article
Language:English
Published: MDPI AG 2019-04-01
Series:Cells
Subjects:
Online Access:https://www.mdpi.com/2073-4409/8/4/379
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author Chaojun Yan
Xiaoying Duanmu
Ling Zeng
Bing Liu
Zhiyin Song
author_facet Chaojun Yan
Xiaoying Duanmu
Ling Zeng
Bing Liu
Zhiyin Song
author_sort Chaojun Yan
collection DOAJ
description 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.
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spelling doaj.art-6c417b6e17b842259b5299d2c82f8dcb2023-09-02T19:47:27ZengMDPI AGCells2073-44092019-04-018437910.3390/cells8040379cells8040379Mitochondrial DNA: Distribution, Mutations, and EliminationChaojun Yan0Xiaoying Duanmu1Ling Zeng2Bing Liu3Zhiyin Song4Hubei Key Laboratory of Cell Homeostasis, College of Life Sciences, Wuhan University, Wuhan 430072, ChinaHubei Key Laboratory of Cell Homeostasis, College of Life Sciences, Wuhan University, Wuhan 430072, ChinaHubei Key Laboratory of Cell Homeostasis, College of Life Sciences, Wuhan University, Wuhan 430072, ChinaHubei Key Laboratory of Cell Homeostasis, College of Life Sciences, Wuhan University, Wuhan 430072, ChinaHubei Key Laboratory of Cell Homeostasis, College of Life Sciences, Wuhan University, Wuhan 430072, ChinaMitochondrion 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.https://www.mdpi.com/2073-4409/8/4/379mitochondriamtDNAmitophagymtDNA distributionmitochondrial dynamics
spellingShingle Chaojun Yan
Xiaoying Duanmu
Ling Zeng
Bing Liu
Zhiyin Song
Mitochondrial DNA: Distribution, Mutations, and Elimination
Cells
mitochondria
mtDNA
mitophagy
mtDNA distribution
mitochondrial dynamics
title Mitochondrial DNA: Distribution, Mutations, and Elimination
title_full Mitochondrial DNA: Distribution, Mutations, and Elimination
title_fullStr Mitochondrial DNA: Distribution, Mutations, and Elimination
title_full_unstemmed Mitochondrial DNA: Distribution, Mutations, and Elimination
title_short Mitochondrial DNA: Distribution, Mutations, and Elimination
title_sort mitochondrial dna distribution mutations and elimination
topic mitochondria
mtDNA
mitophagy
mtDNA distribution
mitochondrial dynamics
url https://www.mdpi.com/2073-4409/8/4/379
work_keys_str_mv AT chaojunyan mitochondrialdnadistributionmutationsandelimination
AT xiaoyingduanmu mitochondrialdnadistributionmutationsandelimination
AT lingzeng mitochondrialdnadistributionmutationsandelimination
AT bingliu mitochondrialdnadistributionmutationsandelimination
AT zhiyinsong mitochondrialdnadistributionmutationsandelimination