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Structure, function, and regulation of mitofusin-2 in health and disease
Biological Reviews ( IF 10.0 ) Pub Date : 2017-10-25 , DOI: 10.1111/brv.12378
Gursimran Chandhok 1 , Michael Lazarou 2 , Brent Neumann 1
Affiliation  

Mitochondria are highly dynamic organelles that constantly migrate, fuse, and divide to regulate their shape, size, number, and bioenergetic function. Mitofusins (Mfn1/2), optic atrophy 1 (OPA1), and dynamin‐related protein 1 (Drp1), are key regulators of mitochondrial fusion and fission. Mutations in these molecules are associated with severe neurodegenerative and non‐neurological diseases pointing to the importance of functional mitochondrial dynamics in normal cell physiology. In recent years, significant progress has been made in our understanding of mitochondrial dynamics, which has raised interest in defining the physiological roles of key regulators of fusion and fission and led to the identification of additional functions of Mfn2 in mitochondrial metabolism, cell signalling, and apoptosis. In this review, we summarize the current knowledge of the structural and functional properties of Mfn2 as well as its regulation in different tissues, and also discuss the consequences of aberrant Mfn2 expression.

中文翻译:

mitofusin-2 在健康和疾病中的结构、功能和调节

线粒体是高度动态的细胞器,不断迁移、融合和分裂以调节其形状、大小、数量和生物能量功能。线粒体融合蛋白 (Mfn1/2)、视神经萎缩 1 (OPA1) 和动力蛋白相关蛋白 1 (Drp1) 是线粒体融合和裂变的关键调节因子。这些分子的突变与严重的神经退行性疾病和非神经系统疾病有关,表明功能性线粒体动力学在正常细胞生理学中的重要性。近年来,我们对线粒体动力学的理解取得了重大进展,这引起了人们对定义融合和裂变关键调节因子的生理作用的兴趣,并导致鉴定了 Mfn2 在线粒体代谢、细胞信号传导和细胞凋亡。在这次审查中,
更新日期:2017-10-25
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