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]
Function:
Copper chaperone for cytochrome c oxidase (COX). Binds two copper ions and deliver them to the Cu(A) site of COX.
Subunit:
Interacts with COA1. Interacts with the chaperone CHCHD4; this is important for correct folding and the formation of disulfide bonds that stabilize the structure.
Subcellular Location:
Mitochondrion intermembrane space.
Tissue Specificity:
Ubiquitous.
Similarity:
Belongs to the COX17 family.
SWISS:
P07327
Gene ID:
10063
Database links:
Entrez Gene: 10063 Human
Entrez Gene: 12856 Mouse
Entrez Gene: 89786 Rat
Omim: 604813 Human
SwissProt: Q14061 Human
SwissProt: P56394 Mouse
Unigene: 534383 Human
Unigene: 27396 Mouse
Unigene: 19207 Rat
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