Mitochondrial dynamics--fusion, fission, movement, and mitophagy--in neurodegenerative diseases.
Mitochondrial dynamics--fusion, fission, movement, and mitophagy--in neurodegenerative diseases.
复制标题
DOI：
10.1093/hmg/ddp326
复制
发表时间：
2009-10-15
影响因子：
3.5
通讯作者：
Chan DC
中科院分区：
文献类型：
--
作者：
Chen H;Chan DC
神经元是代谢活跃的细胞，在远离细胞体的位置具有高能量需求。因此，这些细胞特别依赖于线粒体功能，如通过观察到线粒体功能障碍的疾病通常具有神经退行性成分所反映的。最近的发现强调了神经元特别依赖于线粒体的动态特性。线粒体是动态细胞器的几个标准。它们参与融合和分裂的重复循环，这有助于混合脂质和线粒体群体的内容物。此外，线粒体被积极募集到亚细胞部位，如神经元的轴突和树突过程。最后，线粒体群体的质量通过线粒体自噬来维持，线粒体自噬是一种自噬形式，其中有缺陷的线粒体被选择性地降解。我们回顾了线粒体动力学的一般特征，结合最近的研究结果，线粒体融合，分裂，运输和线粒体自噬。这些关键特征的缺陷与神经退行性疾病有关。Charcot-Marie-Tooth 2A型是一种周围神经病，显性视神经萎缩是一种遗传性视神经病，由线粒体融合的原发性缺陷引起。此外，几种主要的神经退行性疾病--包括帕金森氏症、阿尔茨海默氏症和亨廷顿氏病--都涉及线粒体动力学的破坏。值得注意的是，在几种疾病模型中，线粒体融合或分裂的操作可以部分挽救疾病表型。我们回顾了这些神经退行性疾病中线粒体动力学是如何改变的，并讨论了线粒体融合，分裂，运输和线粒体自噬之间的相互作用。
Neurons are metabolically active cells with high energy demands at locations distant from the cell body. As a result, these cells are particularly dependent on mitochondrial function, as reflected by the observation that diseases of mitochondrial dysfunction often have a neurodegenerative component. Recent discoveries have highlighted that neurons are reliant particularly on the dynamic properties of mitochondria. Mitochondria are dynamic organelles by several criteria. They engage in repeated cycles of fusion and fission, which serve to intermix the lipids and contents of a population of mitochondria. In addition, mitochondria are actively recruited to subcellular sites, such as the axonal and dendritic processes of neurons. Finally, the quality of a mitochondrial population is maintained through mitophagy, a form of autophagy in which defective mitochondria are selectively degraded. We review the general features of mitochondrial dynamics, incorporating recent findings on mitochondrial fusion, fission, transport and mitophagy. Defects in these key features are associated with neurodegenerative disease. Charcot-Marie-Tooth type 2A, a peripheral neuropathy, and dominant optic atrophy, an inherited optic neuropathy, result from a primary deficiency of mitochondrial fusion. Moreover, several major neurodegenerative diseases—including Parkinson's, Alzheimer's and Huntington's disease—involve disruption of mitochondrial dynamics. Remarkably, in several disease models, the manipulation of mitochondrial fusion or fission can partially rescue disease phenotypes. We review how mitochondrial dynamics is altered in these neurodegenerative diseases and discuss the reciprocal interactions between mitochondrial fusion, fission, transport and mitophagy.
登录
查看更多内容
DOI：
10.1083/jcb.200211046
发表时间：
2003-01-20
期刊：
The Journal of cell biology
影响因子：
--
作者：
Chen H;Detmer SA;Ewald AJ;Griffin EE;Fraser SE;Chan DC
通讯作者：
Chan DC
影响因子：
11.8
作者：
Cassidy-Stone, Ann;Chipuk, Jerry E.;Nunnari, Jodi
通讯作者：
Nunnari, Jodi
影响因子：
14.5
作者：
Amati-Bonneau, Patrizia;Valentino, Maria Lucia;Carelli, Valerio
通讯作者：
Carelli, Valerio
影响因子：
64.8
作者：
Clark, Ira E.;Dodson, Mark W.;Guo, Ming
通讯作者：
Guo, Ming
影响因子：
64.8
作者：
de Brito, Olga Martins;Scorrano, Luca
通讯作者：
Scorrano, Luca