Mitochondrial proteases in human diseases,FEBS Letters

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Mitochondrial proteases in human diseases
FEBS Letters ( IF 3.0 ) Pub Date : 2021-02-03 , DOI: 10.1002/1873-3468.14039
Maria Gomez-Fabra Gala _{1,

2,

3} , Friederike-Nora Vögtle _{1,

4}

Affiliation

Mitochondria contain more than 1000 different proteins, including several proteolytic enzymes. These mitochondrial proteases form a complex system that performs limited and terminal proteolysis to build the mitochondrial proteome, maintain and control its functions or degrade mitochondrial proteins and peptides. During protein biogenesis presequence proteases cleave and degrade mitochondrial targeting signals to obtain mature functional proteins. Processing by proteases also exerts a regulatory role in modulation of mitochondrial functions and quality control enzymes degrade misfolded, aged or superfluous proteins. Depending on their different functions and substrates, defects in mitochondrial proteases can affect the majority of the mitochondrial proteome or only a single protein. Consequently, mutations in mitochondrial proteases have been linked to several human diseases. This review gives an overview of the components and functions of the mitochondrial proteolytic machinery and highlights the pathological consequences of dysfunctional mitochondrial protein processing and turnover.

中文翻译：

人类疾病中的线粒体蛋白酶

线粒体包含 1000 多种不同的蛋白质，包括几种蛋白水解酶。这些线粒体蛋白酶形成一个复杂的系统，执行有限的和终末蛋白水解以构建线粒体蛋白质组、维持和控制其功能或降解线粒体蛋白质和肽。在蛋白质生物发生过程中，蛋白酶切割和降解线粒体靶向信号以获得成熟的功能蛋白质。蛋白酶加工还在线粒体功能和质量控制酶的调节中发挥调节作用，降解错误折叠、老化或多余的蛋白质。根据其不同的功能和底物，线粒体蛋白酶的缺陷会影响大部分线粒体蛋白质组或仅影响单个蛋白质。最后，线粒体蛋白酶的突变与多种人类疾病有关。这篇综述概述了线粒体蛋白水解机制的成分和功能，并强调了线粒体蛋白质加工和转换功能障碍的病理后果。

更新日期：2021-02-03

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