COX17 Antibody – Rabbit

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]

COX17: Copper chaperone for cytochrome c oxidase (COX). Binds two copper ions and deliver them to the Cu(A) site of COX. Belongs to the COX17 family.

Protein type: Mitochondrial; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 3q13.33

Cellular Component: mitochondrion; cytoplasm; mitochondrial intermembrane space

Molecular Function: copper ion binding; copper chaperone activity

Biological Process: generation of precursor metabolites and energy; heart development; brain development; copper ion transport