Cytochrome c oxidase subunit III

Available protein structures:
PDB	IPR000298 PF00510 (ECOD; PDBsum)
AlphaFold	IPR000298; PF00510;

Cytochrome c oxidase subunit III
Cytochrome c oxidase subunit III
	Structure of the 13-subunit oxidized cytochrome c oxidase.
Identifiers
Symbol	COX3
Pfam	PF00510
InterPro	IPR000298
PROSITE	PDOC50253
SCOP2	1occ / SCOPe / SUPFAM
TCDB	3.D.4
OPM superfamily	4
OPM protein	1v55
CDD	cd01665
PDB
Available protein structures:
PDB	IPR000298 PF00510 (ECOD; PDBsum)
AlphaFold	IPR000298; PF00510;

COX3
Identifiers
Aliases	COX3, COIII, MTCO3, Cytochrome c oxidase subunit III, cytochrome c oxidase III
External IDs	OMIM: 516050; MGI: 102502; HomoloGene: 5014; GeneCards: COX3; OMA:COX3 - orthologs
Gene location (Human)
Chr.	Mitochondrial DNA (human)
End	9,990 bp
Gene location (Mouse)
Chr.	Mitochondrial DNA (mouse)
End	9,390 bp
RNA expression pattern
	Top expressed in
	gastric mucosa; ; skin of abdomen; ; skin of leg; ; left testis; ; right testis; ; mucosa of transverse colon; ; apex of heart; ; olfactory zone of nasal mucosa; ; amygdala; ; hippocampus proper;
	Top expressed in
	quadriceps femoris muscle; ; right kidney; ; proximal tubule; ; duodenum; ; epiblast; ; ventricular zone; ; ganglionic eminence; ; zone of skin; ; jejunum; ; bone marrow;
	More reference expression data
	n/a
Gene ontology
Molecular function	cytochrome-c oxidase activity; protein binding; electron transfer activity; oxidoreduction-driven active transmembrane transporter activity;
Cellular component	integral component of membrane; mitochondrial inner membrane; respiratory chain complex IV; membrane; mitochondrion; mitochondrial respiratory chain complex IV;
Biological process	proton transmembrane transport; aerobic electron transport chain; respiratory chain complex IV assembly; respiratory electron transport chain; mitochondrial electron transport, cytochrome c to oxygen; aerobic dissimilation; transmembrane transport;
	Sources:Amigo / QuickGO
Orthologs
	4514
	17710
	ENSG00000198938
	ENSMUSG00000064358
	P00414
	P00416
	n/a
	n/a
	n/a
	NP_904334
	Wikidata
View/Edit Human	View/Edit Mouse

Cytochrome c oxidase subunit III (COX3) is an enzyme that in humans is encoded by the MT-CO3 gene. It is one of main transmembrane subunits of cytochrome c oxidase. It is also one of the three mitochondrial DNA (mtDNA) encoded subunits (MT-CO1, MT-CO2, MT-CO3) of respiratory complex IV. Variants of it have been associated with isolated myopathy, severe encephalomyopathy, Leber hereditary optic neuropathy, mitochondrial complex IV deficiency, and recurrent myoglobinuria .