Mitochondrial protein dysfunction in pathogenesis of neurological diseases

Mitochondria are essential organelles for neuronal function and cell survival. Besides the well-known bioenergetics, additional mitochondrial roles in calcium signaling, lipid biogenesis, regulation of reactive oxygen species, and apoptosis are pivotal in diverse cellular processes. The mitochondria...

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Main Authors: Liang Wang, Ziyun Yang, Xiumei He, Shiming Pu, Cheng Yang, Qiong Wu, Zuping Zhou, Xiaobo Cen, Hongxia Zhao
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-09-01
Series:Frontiers in Molecular Neuroscience
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fnmol.2022.974480/full
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author Liang Wang
Ziyun Yang
Ziyun Yang
Ziyun Yang
Ziyun Yang
Xiumei He
Xiumei He
Xiumei He
Shiming Pu
Shiming Pu
Shiming Pu
Cheng Yang
Cheng Yang
Cheng Yang
Qiong Wu
Qiong Wu
Qiong Wu
Zuping Zhou
Zuping Zhou
Xiaobo Cen
Hongxia Zhao
Hongxia Zhao
Hongxia Zhao
Hongxia Zhao
author_facet Liang Wang
Ziyun Yang
Ziyun Yang
Ziyun Yang
Ziyun Yang
Xiumei He
Xiumei He
Xiumei He
Shiming Pu
Shiming Pu
Shiming Pu
Cheng Yang
Cheng Yang
Cheng Yang
Qiong Wu
Qiong Wu
Qiong Wu
Zuping Zhou
Zuping Zhou
Xiaobo Cen
Hongxia Zhao
Hongxia Zhao
Hongxia Zhao
Hongxia Zhao
author_sort Liang Wang
collection DOAJ
description Mitochondria are essential organelles for neuronal function and cell survival. Besides the well-known bioenergetics, additional mitochondrial roles in calcium signaling, lipid biogenesis, regulation of reactive oxygen species, and apoptosis are pivotal in diverse cellular processes. The mitochondrial proteome encompasses about 1,500 proteins encoded by both the nuclear DNA and the maternally inherited mitochondrial DNA. Mutations in the nuclear or mitochondrial genome, or combinations of both, can result in mitochondrial protein deficiencies and mitochondrial malfunction. Therefore, mitochondrial quality control by proteins involved in various surveillance mechanisms is critical for neuronal integrity and viability. Abnormal proteins involved in mitochondrial bioenergetics, dynamics, mitophagy, import machinery, ion channels, and mitochondrial DNA maintenance have been linked to the pathogenesis of a number of neurological diseases. The goal of this review is to give an overview of these pathways and to summarize the interconnections between mitochondrial protein dysfunction and neurological diseases.
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spelling doaj.art-5964c3fccd474802baa8d9b90c9948172022-12-22T04:28:45ZengFrontiers Media S.A.Frontiers in Molecular Neuroscience1662-50992022-09-011510.3389/fnmol.2022.974480974480Mitochondrial protein dysfunction in pathogenesis of neurological diseasesLiang Wang0Ziyun Yang1Ziyun Yang2Ziyun Yang3Ziyun Yang4Xiumei He5Xiumei He6Xiumei He7Shiming Pu8Shiming Pu9Shiming Pu10Cheng Yang11Cheng Yang12Cheng Yang13Qiong Wu14Qiong Wu15Qiong Wu16Zuping Zhou17Zuping Zhou18Xiaobo Cen19Hongxia Zhao20Hongxia Zhao21Hongxia Zhao22Hongxia Zhao23National Chengdu Center for Safety Evaluation of Drugs, State Key Laboratory of Biotherapy/Collaborative Innovation Center for Biotherapy, West China Hospital of Sichuan University, Chengdu, ChinaNational Chengdu Center for Safety Evaluation of Drugs, State Key Laboratory of Biotherapy/Collaborative Innovation Center for Biotherapy, West China Hospital of Sichuan University, Chengdu, ChinaSchool of Life Sciences, Guangxi Normal University, Guilin, ChinaGuangxi Universities, Key Laboratory of Stem Cell and Biopharmaceutical Technology, Guangxi Normal University, Guilin, ChinaResearch Center for Biomedical Sciences, Guangxi Normal University, Guilin, ChinaSchool of Life Sciences, Guangxi Normal University, Guilin, ChinaGuangxi Universities, Key Laboratory of Stem Cell and Biopharmaceutical Technology, Guangxi Normal University, Guilin, ChinaResearch Center for Biomedical Sciences, Guangxi Normal University, Guilin, ChinaSchool of Life Sciences, Guangxi Normal University, Guilin, ChinaGuangxi Universities, Key Laboratory of Stem Cell and Biopharmaceutical Technology, Guangxi Normal University, Guilin, ChinaResearch Center for Biomedical Sciences, Guangxi Normal University, Guilin, ChinaSchool of Life Sciences, Guangxi Normal University, Guilin, ChinaGuangxi Universities, Key Laboratory of Stem Cell and Biopharmaceutical Technology, Guangxi Normal University, Guilin, ChinaResearch Center for Biomedical Sciences, Guangxi Normal University, Guilin, ChinaSchool of Life Sciences, Guangxi Normal University, Guilin, ChinaGuangxi Universities, Key Laboratory of Stem Cell and Biopharmaceutical Technology, Guangxi Normal University, Guilin, ChinaResearch Center for Biomedical Sciences, Guangxi Normal University, Guilin, ChinaGuangxi Universities, Key Laboratory of Stem Cell and Biopharmaceutical Technology, Guangxi Normal University, Guilin, ChinaResearch Center for Biomedical Sciences, Guangxi Normal University, Guilin, ChinaNational Chengdu Center for Safety Evaluation of Drugs, State Key Laboratory of Biotherapy/Collaborative Innovation Center for Biotherapy, West China Hospital of Sichuan University, Chengdu, ChinaSchool of Life Sciences, Guangxi Normal University, Guilin, ChinaGuangxi Universities, Key Laboratory of Stem Cell and Biopharmaceutical Technology, Guangxi Normal University, Guilin, ChinaResearch Center for Biomedical Sciences, Guangxi Normal University, Guilin, ChinaFaculty of Biological and Environmental Sciences, University of Helsinki, Helsinki, FinlandMitochondria are essential organelles for neuronal function and cell survival. Besides the well-known bioenergetics, additional mitochondrial roles in calcium signaling, lipid biogenesis, regulation of reactive oxygen species, and apoptosis are pivotal in diverse cellular processes. The mitochondrial proteome encompasses about 1,500 proteins encoded by both the nuclear DNA and the maternally inherited mitochondrial DNA. Mutations in the nuclear or mitochondrial genome, or combinations of both, can result in mitochondrial protein deficiencies and mitochondrial malfunction. Therefore, mitochondrial quality control by proteins involved in various surveillance mechanisms is critical for neuronal integrity and viability. Abnormal proteins involved in mitochondrial bioenergetics, dynamics, mitophagy, import machinery, ion channels, and mitochondrial DNA maintenance have been linked to the pathogenesis of a number of neurological diseases. The goal of this review is to give an overview of these pathways and to summarize the interconnections between mitochondrial protein dysfunction and neurological diseases.https://www.frontiersin.org/articles/10.3389/fnmol.2022.974480/fullmitochondrial proteinsneurological diseasespathogenesismitochondrial bioenergeticsmitochondrial dynamicsmitophagy
spellingShingle Liang Wang
Ziyun Yang
Ziyun Yang
Ziyun Yang
Ziyun Yang
Xiumei He
Xiumei He
Xiumei He
Shiming Pu
Shiming Pu
Shiming Pu
Cheng Yang
Cheng Yang
Cheng Yang
Qiong Wu
Qiong Wu
Qiong Wu
Zuping Zhou
Zuping Zhou
Xiaobo Cen
Hongxia Zhao
Hongxia Zhao
Hongxia Zhao
Hongxia Zhao
Mitochondrial protein dysfunction in pathogenesis of neurological diseases
Frontiers in Molecular Neuroscience
mitochondrial proteins
neurological diseases
pathogenesis
mitochondrial bioenergetics
mitochondrial dynamics
mitophagy
title Mitochondrial protein dysfunction in pathogenesis of neurological diseases
title_full Mitochondrial protein dysfunction in pathogenesis of neurological diseases
title_fullStr Mitochondrial protein dysfunction in pathogenesis of neurological diseases
title_full_unstemmed Mitochondrial protein dysfunction in pathogenesis of neurological diseases
title_short Mitochondrial protein dysfunction in pathogenesis of neurological diseases
title_sort mitochondrial protein dysfunction in pathogenesis of neurological diseases
topic mitochondrial proteins
neurological diseases
pathogenesis
mitochondrial bioenergetics
mitochondrial dynamics
mitophagy
url https://www.frontiersin.org/articles/10.3389/fnmol.2022.974480/full
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