COX17

Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes a protein which is not a structural subunit, but may be involved in the recruitment of copper to mitochondria for incorporation into the COX apoenzyme. This protein shares 92% amino acid sequence identity with mouse and rat Cox17 proteins. This gene is no longer considered to be a candidate gene for COX deficiency. A pseudogene COX17P has been found on chromosome 13. [provided by RefSeq, Jul 2008]

细胞色素c氧化酶（COX），线粒体呼吸链的终末组分，催化还原型细胞色素c向氧气的电子转移。该组分由线粒体基因编码的3个催化亚基和由核基因编码的多个结构亚基组成的异源多聚体复合物。线粒体编码的亚基参与电子转移，而核基因编码的亚基可能在复合物的调控和组装中发挥作用。此核基因编码的蛋白质并非结构亚基，但可能参与将铜招募至线粒体以整合入COX辅酶无活性酶的过程。该蛋白质与小鼠和大鼠的Cox17蛋白质具有92%的氨基酸序列同源性。此基因不再被视为COX缺乏症的候选基因。在13号染色体上发现了一种伪基因COX17P。[由RefSeq提供，2008年7月]