Oxidative folding in the mitochondrial intermembrane space: A regulated process important for cell physiology and disease.
Oxidative folding in the mitochondrial intermembrane space: A regulated process important for cell physiology and disease.
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DOI：
10.1016/j.bbamcr.2016.03.023
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发表时间：
2016-06
期刊：
影响因子：
--
通讯作者：
Tokatlidis K
中科院分区：
文献类型：
--
作者：
Chatzi A;Manganas P;Tokatlidis K
线粒体是具有复杂内部结构的基本细胞器，可实现重要的多种功能，包括铁硫簇组装和细胞呼吸。 30 多年来的紧张工作已经确定了关键的蛋白质输入成分以及蛋白质靶向和组装所涉及的途径。最近，氧化折叠被发现是线粒体蛋白质稳态的一种重要机制，而几种人类疾病与该途径有关。我们根据假定的氧化还原调节描述了该途径的分子成分，并总结了这些途径与人类疾病之间联系的现有证据。线粒体是铁硫簇组装和细胞呼吸的细胞中心。最近，MIA 途径与 Fe/S 途径、Ca2+ 摄取和细胞凋亡相关。线粒体以及 ER 和过氧化物酶体是 ROS 的主要来源。许多疾病与线粒体功能障碍有关。
Mitochondria are fundamental organelles with a complex internal architecture that fulfill important diverse functions including iron–sulfur cluster assembly and cell respiration. Intense work for more than 30 years has identified the key protein import components and the pathways involved in protein targeting and assembly. More recently, oxidative folding has been discovered as one important mechanism for mitochondrial proteostasis whilst several human disorders have been linked to this pathway. We describe the molecular components of this pathway in view of their putative redox regulation and we summarize available evidence on the connections of these pathways to human disorders. Mitochondria are the cell center of iron–sulfur cluster assembly and cell respiration. The MIA pathway has recently been linked to Fe/S pathways, Ca2 + uptake and apoptosis. Mitochondria along with the ER and peroxisomes are major sources of ROS. Many diseases have been linked to mitochondrial dysfunction.