Mitochondrial DNA damage and reactive oxygen species in neurodegenerative disease.

Mitochondrial DNA damage and reactive oxygen species in neurodegenerative disease.
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DOI:
10.1002/1873-3468.12956
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发表时间:
2018-03
期刊:
影响因子:
3.5
通讯作者:
Moraes CT
Moraes CT
中科院分区:
生物学3区
文献类型:
--
作者:
Nissanka N;Moraes CT

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线粒体是细胞内必不可少的细胞器,大多数ATP通过氧化磷酸化(OXPHOS)产生。这一过程所需的一部分基因是由线粒体DNA (mtDNA)编码的。OXPHOS的一个后果是产生线粒体活性氧(ROS),其在介导细胞损伤,特别是在衰老过程中损伤mtDNA中的作用一直存在争议。有一些神经元亚群似乎对ros诱导的损伤更敏感,线粒体功能障碍与几种神经退行性疾病有关。在这篇综述中,我们将讨论目前在mtDNA和神经退行性变领域的知识,关于ROS是神经元功能的病理或有益贡献者的争论,真正的mtDNA疾病,以及mtDNA缺陷影响中枢神经系统的小鼠模型的见解。
Mitochondria are essential organelles within the cell where most ATP is produced through oxidative phosphorylation (OXPHOS). A subset of the genes needed for this process are encoded by the mitochondrial DNA (mtDNA). One consequence of OXPHOS is the production of mitochondrial reactive oxygen species (ROS), whose role in mediating cellular damage, particularly in damaging mtDNA during ageing, has been controversial. There are subsets of neurons that appear to be more sensitive to ROS-induced damage, and mitochondrial dysfunction has been associated with several neurodegenerative disorders. In this review, we will discuss the current knowledge in the field of mtDNA and neurodegeneration, the debate about ROS as a pathological or beneficial contributor to neuronal function, bona fide mtDNA diseases, and insights from mouse models of mtDNA defects affecting the central nervous system.
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