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Mitofusins as mitochondrial anchors and tethers.
Journal of Molecular and Cellular Cardiology ( IF 4.9 ) Pub Date : 2020-04-15 , DOI: 10.1016/j.yjmcc.2020.04.016 Gerald W Dorn 1
Journal of Molecular and Cellular Cardiology ( IF 4.9 ) Pub Date : 2020-04-15 , DOI: 10.1016/j.yjmcc.2020.04.016 Gerald W Dorn 1
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Mitochondria have their own genomes and their own agendas. Like their primitive bacterial ancestors, mitochondria interact with their environment and organelle colleagues at their physical interfaces, the outer mitochondrial membrane. Among outer membrane proteins, mitofusins (MFN) are increasingly recognized for their roles as arbiters of mitochondria-mitochondria and mitochondria-reticular interactions. This review examines the roles of MFN1 and MFN2 in the heart and other organs as proteins that tether mitochondria to each other or to other organelles, and as mitochondrial anchoring proteins for various macromolecular complexes. The consequences of MFN-mediated tethering and anchoring on mitochondrial fusion, motility, mitophagy, and mitochondria-ER calcium cross-talk are reviewed. Pathophysiological implications are explored from the perspective of mitofusin common functioning as tethering and anchoring proteins, rather than as mediators of individual processes. Finally, some informed speculation is provided for why mouse MFN knockout studies show severe multi-system phenotypes whereas rare human diseases linked to MFN mutations are limited in scope.
中文翻译：
线粒体融合蛋白作为线粒体锚和系链。
线粒体有自己的基因组和议程。像它们的原始细菌祖先一样，线粒体与周围环境相互作用，并在其物理界面即线粒体外膜上与细胞器同事互动。在外膜蛋白中，线粒体融合蛋白（MFN）因其作为线粒体-线粒体和线粒体-网状相互作用的仲裁者而日益受到认可。这篇综述检查了MFN1和MFN2在心脏和其他器官中的作用，它们是将线粒体彼此束缚或与其他细胞器束缚的蛋白质，以及作为各种高分子复合物的线粒体锚定蛋白质。审查了最惠国待遇介导的绑定和锚定对线粒体融合，运动性，线粒体和线粒体-ER钙串扰的后果。从mitofusin的共同功能为系链和锚定蛋白，而不是作为单个过程的介质，探讨了病理生理学意义。最后，提供了一些有根据的推测，为什么小鼠MFN基因敲除研究显示出严重的多系统表型，而与MFN突变相关的罕见人类疾病却受到局限。
更新日期：2020-04-15
中文翻译：
线粒体融合蛋白作为线粒体锚和系链。
线粒体有自己的基因组和议程。像它们的原始细菌祖先一样，线粒体与周围环境相互作用，并在其物理界面即线粒体外膜上与细胞器同事互动。在外膜蛋白中，线粒体融合蛋白（MFN）因其作为线粒体-线粒体和线粒体-网状相互作用的仲裁者而日益受到认可。这篇综述检查了MFN1和MFN2在心脏和其他器官中的作用，它们是将线粒体彼此束缚或与其他细胞器束缚的蛋白质，以及作为各种高分子复合物的线粒体锚定蛋白质。审查了最惠国待遇介导的绑定和锚定对线粒体融合，运动性，线粒体和线粒体-ER钙串扰的后果。从mitofusin的共同功能为系链和锚定蛋白，而不是作为单个过程的介质，探讨了病理生理学意义。最后，提供了一些有根据的推测，为什么小鼠MFN基因敲除研究显示出严重的多系统表型，而与MFN突变相关的罕见人类疾病却受到局限。
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