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线粒体融合蛋白2:线粒体功能与神经退行性变之间的功能转换

Mitofusin-2: Functional switch between mitochondrial function and neurodegeneration.

作者信息

Kulkarni Prakash G, Mohire Vaibhavi M, Bhaisa Pooja K, Joshi Mrudula M, Puranik Chitranshi M, Waghmare Pranjal P, Banerjee Tanushree

机构信息

Department of Biotechnology, Savitribai Phule Pune University, Ganeshkhind Road, Pune 411007, India.

Molecular Neuroscience Research Centre, Dr. D. Y. Patil Biotechnology & Bioinformatics Institute, Dr. D. Y. Patil Vidyapeeth Survey No 87/88, Mumbai Bangalore Express Highway, Tathawade, Pune 411 033, India.

出版信息

Mitochondrion. 2023 Mar;69:116-129. doi: 10.1016/j.mito.2023.02.001. Epub 2023 Feb 9.

DOI:10.1016/j.mito.2023.02.001
PMID:36764501
Abstract

Mitochondria are highly dynamic organelles known to play role in the regulation of several cellular biological processes. However, their dynamics such as number, shape, and biological functions are regulated by mitochondrial fusion and fission process. The balance between the fusion and fission process is most important for the maintenance of mitochondrial structure as well as cellular functions. The alterations within mitochondrial dynamic processes were found to be associated with the progression of neurodegenerative diseases. In recent years, mitofusin-2 (Mfn2), a GTPase has emerged as a multifunctional protein which not only is found to regulate the mitochondrial fusion-fission process but also known to regulate several cellular functions such as mitochondrial metabolism, cellular biogenesis, signalling, and apoptosis via maintaining the ER-mitochondria contact sites. In this review, we summarize the current knowledge of the structural and functional properties of the Mfn2, its transcriptional regulation and their roles in several cellular functions with a focus on current advances in the pathogenesis of neurodegenerative diseases.

摘要

线粒体是高度动态的细胞器,已知其在多种细胞生物学过程的调节中发挥作用。然而,它们的动态变化,如数量、形状和生物学功能,是由线粒体融合和裂变过程调节的。融合和裂变过程之间的平衡对于维持线粒体结构以及细胞功能最为重要。人们发现线粒体动态过程中的改变与神经退行性疾病的进展有关。近年来,一种名为线粒体融合蛋白2(Mfn2)的GTP酶已成为一种多功能蛋白,它不仅被发现可调节线粒体融合-裂变过程,还通过维持内质网-线粒体接触位点来调节多种细胞功能,如线粒体代谢、细胞生物合成、信号传导和细胞凋亡。在这篇综述中,我们总结了目前关于Mfn2的结构和功能特性、其转录调控以及它们在多种细胞功能中的作用的知识,重点关注神经退行性疾病发病机制的当前进展。

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