Cytochrome c oxidase subunit I

Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

Cytochrome c oxidase subunit I
Cytochrome c oxidase subunit I
	Structure of the 13-subunit oxidized cytochrome c oxidase.
Identifiers
Symbol	COX1 or COI
Pfam	PF00115
InterPro	IPR000883
PROSITE	PDOC00074
SCOP2	1occ / SCOPe / SUPFAM
TCDB	3.D.4
OPM superfamily	4
OPM protein	1v55
CDD	cd01663
Pfam
Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

COX1
Identifiers
Aliases	COX1, mitochondrially encoded cytochrome c oxidase I, COI, MTCO1, Main subunit of cytochrome c oxidase, CO I, cytochrome c oxidase subunit I
External IDs	OMIM: 516030 MGI: 102504 HomoloGene: 5016 GeneCards: COX1
Gene location (Human)
Chr.	Mitochondrial DNA (human)
End	7,445 bp
Gene location (Mouse)
Chr.	Mitochondrial DNA (mouse)
End	6,872 bp
RNA expression pattern
	Top expressed in
	right uterine tube; ; duodenum; ; stromal cell of endometrium; ; right coronary artery; ; rectum; ; Brodmann area 9; ; smooth muscle tissue; ; gallbladder; ; appendix; ; right lung;
	Top expressed in
	cerebellar cortex; ; esophagus; ; superior frontal gyrus; ; hippocampus proper; ; jejunum; ; ganglionic eminence; ; quadriceps femoris muscle; ; kidney; ; lip; ; colon;
	More reference expression data
	n/a
Gene ontology
Molecular function	cytochrome-c oxidase activity; metal ion binding; protein binding; heme binding; oxidoreductase activity;
Cellular component	integral component of membrane; membrane; mitochondrion; mitochondrial inner membrane; respirasome; respiratory chain complex IV; mitochondrial respiratory chain complex III; mitochondrial respiratory chain complex IV;
Biological process	response to copper ion; human ageing; response to electrical stimulus; response to oxidative stress; cerebellum development; oxidative phosphorylation; mitochondrial electron transport, cytochrome c to oxygen; aerobic respiration; electron transport coupled proton transport;
	Sources:Amigo / QuickGO
Orthologs
	4512
	17708
	ENSG00000198804
	ENSMUSG00000064351
	P00395
	P00397
	n/a
	n/a
	n/a
	NP_904330
	Wikidata
View/Edit Human	View/Edit Mouse

Cytochrome c oxidase I (COX1) also known as mitochondrially encoded cytochrome c oxidase I (MT-CO1) is a protein that is encoded by the MT-CO1 gene in eukaryotes. The gene is also called COX1, CO1, or COI. Cytochrome c oxidase I is the main subunit of the cytochrome c oxidase complex. In humans, mutations in MT-CO1 have been associated with Leber's hereditary optic neuropathy (LHON), acquired idiopathic sideroblastic anemia, Complex IV deficiency, colorectal cancer, sensorineural deafness, and recurrent myoglobinuria.

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