Neurodegeneration-associated mitochondrial proteins, CHCHD2 and CHCHD10-what distinguishes the two?

Neurodegeneration-associated mitochondrial proteins, CHCHD2 and CHCHD10-what distinguishes the two?
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神经变性相关的线粒体蛋白,chchd2和chchd10-有什么区别两者?

DOI:
10.3389/fcell.2022.996061
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发表时间:
2022
影响因子:
5.5
通讯作者:
--
中科院分区:
生物学2区
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--
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螺旋结构域2(CHCHD2)和螺旋结构域10(CHCHD10)是线粒体蛋白质,被认为是在进化过程中复制的基因,分别是帕金森病和肌萎缩侧索硬化症/额颞叶痴呆的致病基因。CHCHD2与CHCHD10形成异源二聚体,与自身形成同源二聚体,两者在线粒体内协同工作。已经确定了各种致病和致病风险变异;然而,这些突变如何在特定疾病中导致神经退化仍然是一个谜。这篇综述重点介绍了自2019年以来发表的重要新发现,并讨论了解开这一谜团的途径。
Coiled-coil-helix-coiled-coil-helix domain containing 2 (CHCHD2) and Coiled-coil-helix-coiled-coil-helix domain containing 10 (CHCHD10) are mitochondrial proteins that are thought to be genes which duplicated during evolution and are the causative genes for Parkinson’s disease and amyotrophic lateral sclerosis/frontotemporal lobe dementia, respectively. CHCHD2 forms a heterodimer with CHCHD10 and a homodimer with itself, both of which work together within the mitochondria. Various pathogenic and disease-risk variants have been identified; however, how these mutations cause neurodegeneration in specific diseases remains a mystery. This review focuses on important new findings published since 2019 and discusses avenues to solve this mystery.
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