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Mitochondrial Fusion: The Machineries In and Out
Trends in Cell Biology ( IF 13.0 ) Pub Date : 2020-10-19 , DOI: 10.1016/j.tcb.2020.09.008
Song Gao 1 , Junjie Hu 2
Affiliation  

Mitochondria are highly dynamic organelles that constantly undergo fission and fusion. Disruption of mitochondrial dynamics undermines their function and causes several human diseases. The fusion of the outer (OMM) and inner mitochondrial membranes (IMM) is mediated by two classes of dynamin-like protein (DLP): mitofusin (MFN)/fuzzy onions 1 (Fzo1) and optic atrophy 1/mitochondria genome maintenance 1 (OPA1/Mgm1). Given the lack of structural information on these fusogens, the molecular mechanisms underlying mitochondrial fusion remain unclear, even after 20 years. Here, we review recent advances in structural studies of the mitochondrial fusion machinery, discuss their implication for DLPs, and summarize the pathogenic mechanisms of disease-causing mutations in mitochondrial fusion DLPs.



中文翻译:

线粒体融合:进进出出的机器

线粒体是高度动态的细胞器,不断进行裂变和融合。线粒体动力学的中断会破坏其功能并导致多种人类疾病。线粒体外膜 (OMM) 和线粒体内膜 (IMM) 的融合由两类动力蛋白 (DLP) 介导:线粒体融合蛋白 (MFN)/模糊洋葱 1 (Fzo1) 和视神经萎缩 1/线粒体基因组维持 1 ( OPA1/Mgm1)。鉴于缺乏关于这些融合素的结构信息,即使在 20 年后,线粒体融合的分子机制仍不清楚。在这里,我们回顾了线粒体融合机制结构研究的最新进展,讨论了它们对 DLP 的影响,并总结了线粒体融合 DLP 中致病突变的致病机制。

更新日期:2020-12-17
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