The Erv1-Mia40 disulfide relay system in the intermembrane space of mitochondria
The Erv1-Mia40 disulfide relay system in the intermembrane space of mitochondria
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DOI：
10.1016/j.bbamcr.2007.12.005
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发表时间：
2008-04-01
影响因子：
5.1
通讯作者：
Hell, Kai
中科院分区：
文献类型：
--
作者：
Hell, Kai
线粒体外膜和内膜之间的隔室，即膜间隙 (IMS)，含有多种含有二硫键的蛋白质。这些蛋白质中的许多都具有保守的孪生 Cx(3)C 基序或孪生 Cx(9)C 基序。最近，IMS 中的二硫键中继系统已被鉴定，该系统由两个基本组件组成：巯基氧化酶 Erv1 和氧化还原调节的输入受体 Mia40/Tim40。二硫键中继系统通过氧化折叠机制驱动这些富含半胱氨酸的蛋白质进入线粒体的IMS。为了使 Mia40 能够进行底物蛋白的氧化，巯基氧化酶 Erv1 在二硫键转移反应中介导 Mia40 的氧化。为了将 Erv1 回收成氧化形式，电子被转移到细胞色素 c，将二硫键中继系统连接到线粒体的电子传输链。尽管各组分缺乏同源性，IMS 中的二硫键中继系统类似于细菌周质中的氧化系统，这可能反映了 IMS 从细菌周质的进化起源。 (C) 2007 Elsevier B.V. 保留所有权利。
The compartment between the outer and the inner membranes of mitochondria, the intermembrane space (IMS), harbours a variety of proteins that contain disulfide bonds. Many of these proteins possess a conserved twin Cx(3)C motif or twin Cx(9)C motif Recently, a disulfide relay system in the IMS has been identified which consists of two essential components, the sulfhydryl oxidase Erv1 and the redox-regulated import receptor Mia40/Tim40. The disulfide relay system drives the import of these cysteine-rich proteins into the IMS of mitochondria by an oxidative folding mechanism. In order to enable Mia40 to perform the oxidation of substrate proteins, the sulfhydryl oxidase Erv1 mediates the oxidation of Mia40 in a disulfide transfer reaction. To recycle Erv1 into its oxidized form, electrons are transferred to cytochrome c connecting the disulfide relay system to the electron transport chain of mitochondria. Despite the lack of homology of the components, the disulfide relay system in the IMS resembles the oxidation system in the periplasm of bacteria presumably reflecting the evolutionary origin of the IMS from the bacterial periplasm. (C) 2007 Elsevier B.V. All rights reserved.