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; OMA:COX1 - orthologs
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; ; apex of heart; ; duodenum; ; stromal cell of endometrium; ; right coronary artery; ; rectum; ; Brodmann area 9; ; smooth muscle tissue; ; gallbladder; ; appendix;
	Top expressed in
	dentate gyrus of hippocampal formation granule cell; ; cerebellar cortex; ; esophagus; ; primary visual cortex; ; Cortex of frontal lobe; ; superior frontal gyrus; ; hippocampus proper; ; muscle of thigh; ; jejunum; ; ganglionic eminence;
	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; 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.^[6] The gene is also called COX1, CO1, or COI.^[7] 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.^[8]^[9]^[10]

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000198804 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000064351 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ Tsukihara T, Aoyama H, Yamashita E, Tomizaki T, Yamaguchi H, Shinzawa-Itoh K, et al. (May 1996). "The whole structure of the 13-subunit oxidized cytochrome c oxidase at 2.8 A". Science. 272 (5265): 1136–1144. Bibcode:1996Sci...272.1136T. doi:10.1126/science.272.5265.1136. PMID 8638158. S2CID 20860573.
^ "Entrez Gene: Cytochrome c oxidase subunit I".
^ Kosakyan A, Heger TJ, Leander BS, Todorov M, Mitchell EA, Lara E (May 2012). "COI barcoding of Nebelid testate amoebae (Amoebozoa: Arcellinida): extensive cryptic diversity and redefinition of the Hyalospheniidae Schultze" (PDF). Protist. 163 (3): 415–434. doi:10.1016/j.protis.2011.10.003. PMID 22130576.
^ Cite error: The named reference :1 was invoked but never defined (see the help page).
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[refGRCh38Ensembl-1] GRCh38: Ensembl release 89: ENSG00000198804 – Ensembl, May 2017

[refGRCm38Ensembl-2] GRCm38: Ensembl release 89: ENSMUSG00000064351 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid8638158-5] Tsukihara T, Aoyama H, Yamashita E, Tomizaki T, Yamaguchi H, Shinzawa-Itoh K, et al. (May 1996). "The whole structure of the 13-subunit oxidized cytochrome c oxidase at 2.8 A". Science. 272 (5265): 1136–1144. Bibcode:1996Sci...272.1136T. doi:10.1126/science.272.5265.1136. PMID 8638158. S2CID 20860573.

[entrez-6] "Entrez Gene: Cytochrome c oxidase subunit I".

[pmid22130576-7] Kosakyan A, Heger TJ, Leander BS, Todorov M, Mitchell EA, Lara E (May 2012). "COI barcoding of Nebelid testate amoebae (Amoebozoa: Arcellinida): extensive cryptic diversity and redefinition of the Hyalospheniidae Schultze" (PDF). Protist. 163 (3): 415–434. doi:10.1016/j.protis.2011.10.003. PMID 22130576.

[:1-8] Cite error: The named reference :1 was invoked but never defined (see the help page).

[:0-9] Cite error: The named reference :0 was invoked but never defined (see the help page).

[:3-10] Cite error: The named reference :3 was invoked but never defined (see the help page).

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