线粒体蛋白质导入的质量控制机制。

IF 4.2 2区 医学 Q1 ENDOCRINOLOGY & METABOLISM
Lion Borgert, Thomas Becker, Fabian den Brave
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引用次数: 0

摘要

线粒体承担着细胞的重要功能,包括产生能量、各种生物合成途径、形成辅助因子以及在细胞凋亡和炎症中传递细胞信号。在面包酵母和人类细胞中,线粒体的功能分别需要从细胞质中输入约 900-1300 种蛋白质。这些蛋白质中的绝大多数通过线粒体外膜(TOM)复合体的转运酶,以基本未折叠的状态通过外膜。随后,特定的蛋白质转运酶将前体蛋白质分拣到外膜、内膜、膜间隙和基质中。线粒体前体蛋白折叠过早、线粒体蛋白转运酶缺陷或线粒体内膜膜电位降低都会导致前体蛋白在蛋白导入装置处停滞。结果,转译接头被堵塞,未导入的前体蛋白质在细胞内积聚,进而导致蛋白质毒性应激,最终导致细胞死亡。为防止出现这种应激情况,质量控制机制可将非导入前体蛋白从 TOM 通道中清除。细胞质中高度保守的泛素-蛋白酶体系统在这一过程中发挥着关键作用。因此,通过 TOM 复合物对蛋白质导入的监控涉及线粒体定位蛋白和细胞质蛋白的协调活动,以防止细胞中出现蛋白毒性压力。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Conserved quality control mechanisms of mitochondrial protein import

Conserved quality control mechanisms of mitochondrial protein import

Mitochondria carry out essential functions for the cell, including energy production, various biosynthesis pathways, formation of co-factors and cellular signalling in apoptosis and inflammation. The functionality of mitochondria requires the import of about 900–1300 proteins from the cytosol in baker's yeast Saccharomyces cerevisiae and human cells, respectively. The vast majority of these proteins pass the outer membrane in a largely unfolded state through the translocase of the outer mitochondrial membrane (TOM) complex. Subsequently, specific protein translocases sort the precursor proteins into the outer and inner membranes, the intermembrane space and matrix. Premature folding of mitochondrial precursor proteins, defects in the mitochondrial protein translocases or a reduction of the membrane potential across the inner mitochondrial membrane can cause stalling of precursors at the protein import apparatus. Consequently, the translocon is clogged and non-imported precursor proteins accumulate in the cell, which in turn leads to proteotoxic stress and eventually cell death. To prevent such stress situations, quality control mechanisms remove non-imported precursor proteins from the TOM channel. The highly conserved ubiquitin-proteasome system of the cytosol plays a critical role in this process. Thus, the surveillance of protein import via the TOM complex involves the coordinated activity of mitochondria-localized and cytosolic proteins to prevent proteotoxic stress in the cell.

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来源期刊
Journal of Inherited Metabolic Disease
Journal of Inherited Metabolic Disease 医学-内分泌学与代谢
CiteScore
9.50
自引率
7.10%
发文量
117
审稿时长
4-8 weeks
期刊介绍: The Journal of Inherited Metabolic Disease (JIMD) is the official journal of the Society for the Study of Inborn Errors of Metabolism (SSIEM). By enhancing communication between workers in the field throughout the world, the JIMD aims to improve the management and understanding of inherited metabolic disorders. It publishes results of original research and new or important observations pertaining to any aspect of inherited metabolic disease in humans and higher animals. This includes clinical (medical, dental and veterinary), biochemical, genetic (including cytogenetic, molecular and population genetic), experimental (including cell biological), methodological, theoretical, epidemiological, ethical and counselling aspects. The JIMD also reviews important new developments or controversial issues relating to metabolic disorders and publishes reviews and short reports arising from the Society''s annual symposia. A distinction is made between peer-reviewed scientific material that is selected because of its significance for other professionals in the field and non-peer- reviewed material that aims to be important, controversial, interesting or entertaining (“Extras”).
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