MitoProteome Database

MT000748

Record overview

MITO IDMT000748
Gene ID10063
SpeciesHomo sapiens (Human)
Gene NameCOX17 cytochrome c oxidase copper chaperone
Gene SymbolCOX17
Alternate namescytochrome c oxidase copper chaperone; cytochrome c oxidase 17 copper chaperone; cytochrome c oxidase assembly homolog 17; COX17 cytochrome c oxidase assembly homolog; human homolog of yeast mitochondrial copper recruitment;
Chromosome3
Map Location3q13.33
SummaryCytochrome 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]
OrthologsView orthologs and multiple alignments for COX17

Proteins

cytochrome c oxidase copper chaperone
Refseq ID:NP_005685
Protein GI:5031645
UniProt ID:Q14061
mRNA ID:NM_005694
Length:63
RefSeq Status:
MPGLVDSNPAPPESQEKKPLKPCCACPETKKARDACIIEKGEEHCGHLIEAHKECMRALGFKI