Getting out what you put in: Copper in mitochondria and its impacts on human disease.
Getting out what you put in: Copper in mitochondria and its impacts on human disease.
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拿出您的内容：线粒体中的铜及其对人类疾病的影响。
DOI：
10.1016/j.bbamcr.2020.118867
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发表时间：
2021-01
期刊：
影响因子：
--
通讯作者：
Leary SC
中科院分区：
文献类型：
--
作者：
Cobine PA;Moore SA;Leary SC
线粒体在其基质中积累铜，用于铜酶细胞色素c氧化酶和超氧化物歧化酶的最终成熟。通过线粒体载体家族（MCF）蛋白实现向基质中的转运。迄今为止在酵母中描述的主要铜转运MCF是Pic2，其将金属离子输入基质中。Pic2是约30种MCFs之一，其将许多代谢物、核苷酸和辅因子移动穿过内膜以用于基质中。遗传和生化实验表明，Pic2是所需的细胞色素c氧化酶活性在铜胁迫下，它是能够运输离子和络合形式的铜。Pic2直系同源物SLC25A3是53种哺乳动物MCFs之一，具有铜和磷酸盐转运蛋白的功能。SLC25A3的耗尽导致铜在基质中的积累减少，细胞色素c氧化酶缺陷和细胞溶质超氧化物歧化酶丰度的调节。铜和铜蛋白在细胞器中的调节作用继续扩展到细胞器之外。线粒体铜分子伴侣与细胞铜摄取和输出的调节以及器官间通讯的促进有关。最近，还提出了基质铜在一种称为铜中毒的新型细胞死亡途径中的作用。本文将详细介绍线粒体铜酶的成熟、线粒体信号在调节细胞内铜含量中的作用、铜转运到细胞器中的可能机制以及铜稳态途径的进化起源。
Mitochondria accumulate copper in their matrix for the eventual maturation of the cuproenzymes cytochrome c oxidase and superoxide dismutase. Transport into the matrix is achieved by mitochondrial carrier family (MCF) proteins. The major copper transporting MCF described to date in yeast is Pic2, which imports the metal ion into the matrix. Pic2 is one of ~30 MCFs that move numerous metabolites, nucleotides and co-factors across the inner membrane for use in the matrix. Genetic and biochemical experiments showed that Pic2 is required for cytochrome c oxidase activity under copper stress, and that it is capable of transporting ionic and complexed forms of copper. The Pic2 ortholog SLC25A3, one of 53 mammalian MCFs, functions as both a copper and a phosphate transporter. Depletion of SLC25A3 results in decreased accumulation of copper in the matrix, a cytochrome c oxidase defect and a modulation of cytosolic superoxide dismutase abundance. The regulatory roles for copper and cuproproteins resident to the mitochondrion continue to expand beyond the organelle. Mitochondrial copper chaperones have been linked to the modulation of cellular copper uptake and export and the facilitation of inter-organ communication. Recently, a role for matrix copper has also been proposed in a novel cell death pathway termed cuproptosis. This review will detail our understanding of the maturation of mitochondrial copper enzymes, the roles of mitochondrial signals in regulating cellular copper content, the proposed mechanisms of copper transport into the organelle and explore the evolutionary origins of copper homeostasis pathways.
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影响因子：
6.4
作者：
Garcia-Santamarina S;Uzarska MA;Festa RA;Lill R;Thiele DJ
通讯作者：
Thiele DJ
影响因子：
15
作者：
Dodani, Sheel C.;Leary, Scot C.;Cobine, Paul A.;Winge, Dennis R.;Chang, Christopher J.
通讯作者：
Chang, Christopher J.
影响因子：
4.8
作者：
Beers, J;Glerum, DM;Tzagoloff, A
通讯作者：
Tzagoloff, A
影响因子：
4.8
作者：
Cobine, Paul A.;Pierrel, Fabien;Winge, Dennis R.
通讯作者：
Winge, Dennis R.
DOI：
10.1073/pnas.0800019105
发表时间：
2008-05-13
影响因子：
11.1
作者：
Banci, Lucia;Bertini, Ivano;Palumaa, Peep
通讯作者：
Palumaa, Peep