Mechanisms of Mitochondrial Iron-Sulfur Protein Biogenesis.

IF 12.1 1区 生物学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY
Annual review of biochemistry Pub Date : 2020-06-20 Epub Date: 2020-01-14 DOI:10.1146/annurev-biochem-013118-111540
Roland Lill, Sven-A Freibert
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引用次数: 170

Abstract

Mitochondria are essential in most eukaryotes and are involved in numerous biological functions including ATP production, cofactor biosyntheses, apoptosis, lipid synthesis, and steroid metabolism. Work over the past two decades has uncovered the biogenesis of cellular iron-sulfur (Fe/S) proteins as the essential and minimal function of mitochondria. This process is catalyzed by the bacteria-derived iron-sulfur cluster assembly (ISC) machinery and has been dissected into three major steps: de novo synthesis of a [2Fe-2S] cluster on a scaffold protein; Hsp70 chaperone-mediated trafficking of the cluster and insertion into [2Fe-2S] target apoproteins; and catalytic conversion of the [2Fe-2S] into a [4Fe-4S] cluster and subsequent insertion into recipient apoproteins. ISC components of the first two steps are also required for biogenesis of numerous essential cytosolic and nuclear Fe/S proteins, explaining the essentiality of mitochondria. This review summarizes the molecular mechanisms underlying the ISC protein-mediated maturation of mitochondrial Fe/S proteins and the importance for human disease.

线粒体铁硫蛋白生物生成机制。
线粒体在大多数真核生物中都是必不可少的,它参与了许多生物功能,包括产生 ATP、辅助因子生物合成、细胞凋亡、脂质合成和类固醇代谢。过去二十年的研究发现,细胞铁硫(Fe/S)蛋白的生物生成是线粒体最基本的功能。这一过程由细菌衍生的铁硫簇组装(ISC)机制催化,并被分解为三个主要步骤:在支架蛋白上从头合成[2Fe-2S]簇;Hsp70伴侣介导簇的运输并插入[2Fe-2S]目标载体蛋白;以及催化[2Fe-2S]转化为[4Fe-4S]簇并随后插入受体载体蛋白。前两个步骤的 ISC 成分也是许多重要的细胞膜和核铁/核蛋白质生物生成所必需的,这也解释了线粒体的重要性。本综述总结了 ISC 蛋白介导的线粒体 Fe/S 蛋白成熟的分子机制及其对人类疾病的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Annual review of biochemistry
Annual review of biochemistry 生物-生化与分子生物学
CiteScore
33.90
自引率
0.00%
发文量
31
期刊介绍: The Annual Review of Biochemistry, in publication since 1932, sets the standard for review articles in biological chemistry and molecular biology. Since its inception, these volumes have served as an indispensable resource for both the practicing biochemist and students of biochemistry.
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