Cobine Paul A, Pierrel Fabien, Winge Dennis R
Departments of Medicine and Biochemistry, University of Utah Health Sciences Center, Salt Lake City, UT 84132, USA.
Biochim Biophys Acta. 2006 Jul;1763(7):759-72. doi: 10.1016/j.bbamcr.2006.03.002. Epub 2006 Mar 31.
Copper is required within the mitochondrion for the function of two metalloenzymes, cytochrome c oxidase (CcO) and superoxide dismutase (Sod1). Copper metallation of these two enzymes occurs within the mitochondrial intermembrane space and is mediated by metallochaperone proteins. Cox17 is a key copper donor to two accessory proteins, Sco1 and Cox11, to form the two copper centers in the mature CcO complex. Ccs1 is the necessary metallochaperone for the copper metallation of Sod1 in the IMS as well as within the cytoplasm where the bulk of Sod1 resides. Copper ions used in the metallation of CcO and Sod1 appear to be provided by a novel copper pool within the mitochondrial matrix. This review documents copper ion shuttling within the mitochondrion and the proteins that mediate assembly of active CcO and Sod1.
线粒体功能需要铜来维持两种金属酶，即细胞色素c氧化酶（CcO）和超氧化物歧化酶（Sod1）的活性。这两种酶的铜金属化过程发生在线粒体内膜间隙，由金属伴侣蛋白介导。Cox17是两种辅助蛋白Sco1和Cox11的关键铜供体，在成熟的CcO复合物中形成两个铜中心。Ccs1是Sod1在膜间隙以及大部分Sod1所在的细胞质中进行铜金属化所必需的金属伴侣。用于CcO和Sod1金属化的铜离子似乎由线粒体基质中的一个新型铜池提供。本文综述了线粒体内铜离子的穿梭以及介导活性CcO和Sod1组装的蛋白质。
