MT-CO1

Cytochrome c oxidase subunit I
PDB rendering based on 1occ.
Available structures PDB 1occ, 1oco, 1ocr, 1ocz, 1v54, 1v55, 2dyr, 2dys, 2eij, 2eik, 2eil, 2eim, 2ein, 2occ
Identifiers
Symbols	COX1; COI; MTCO1
External IDs	OMIM: 516030 MGI: 102504 HomoloGene: 5016 GeneCards: COX1 Gene
Gene Ontology Molecular function • cytochrome-c oxidase activity • protein binding • electron carrier activity • oxidoreductase activity • heme binding • metal ion binding Cellular component • mitochondrion • mitochondrial inner membrane • membrane • integral to membrane • respiratory chain Biological process • mitochondrial electron transport, cytochrome c to oxygen • transport • response to oxidative stress • aging • aerobic respiration • cerebellum development • respiratory electron transport chain • response to copper ion • response to electrical stimulus Sources: Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	4512	17708
Ensembl	ENSG00000198804	ENSMUSG00000064351
UniProt	P00395	Q35042
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	YP_003024028.1	NP_904330.1
Location (UCSC)	Chr MT: 0.01 – 0.01 Mb	Chr MT: 0.01 – 0.01 Mb
PubMed search	[1]	[2]

"Cox1" redirects here. Particularly in a medical context, this can also refer to cyclooxygenase-1 (COX-1).

Mitochondrially encoded cytochrome c oxidase I (MT-CO1), also known as cytochrome c oxidase I (COX1), is a protein that is found in mitochondria. In humans^{[verification needed]}, the gene encoding it is named mt-co1. In other eukaryotes, the gene is called cox1.

MT-CO1 belongs to cytochrome c oxidase subunit I protein family.^[1] It is often used in DNA barcoding, because its mutation rate is often fast enough to distinguish closely related species. In most if not all seed plants, however, the rate of evolution of cox1 is very slow.

References

1. ^ "Entrez Gene: COX1 cytochrome c oxidase subunit I". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=4512. 

Further reading

• Torroni A, Achilli A, Macaulay V, et al. (2006). "Harvesting the fruit of the human mtDNA tree". Trends Genet. 22 (6): 339–45. doi:10.1016/j.tig.2006.04.001. PMID 16678300. 
• Bodenteich A, Mitchell LG, Polymeropoulos MH, Merril CR (1993). "Dinucleotide repeat in the human mitochondrial D-loop". Hum. Mol. Genet. 1 (2): 140. doi:10.1093/hmg/1.2.140-a. PMID 1301157. 
• Brown MD, Yang CC, Trounce I, et al. (1992). "A mitochondrial DNA variant, identified in Leber hereditary optic neuropathy patients, which extends the amino acid sequence of cytochrome c oxidase subunit I". Am. J. Hum. Genet. 51 (2): 378–85. PMC 1682694. PMID 1322638. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1682694. 
• Lu X, Walker T, MacManus JP, Seligy VL (1992). "Differentiation of HT-29 human colonic adenocarcinoma cells correlates with increased expression of mitochondrial RNA: effects of trehalose on cell growth and maturation". Cancer Res. 52 (13): 3718–25. PMID 1377597. 
• Marzuki S, Noer AS, Lertrit P, et al. (1992). "Normal variants of human mitochondrial DNA and translation products: the building of a reference data base". Hum. Genet. 88 (2): 139–45. PMID 1757091. 
• Moraes CT, Andreetta F, Bonilla E, et al. (1991). "Replication-competent human mitochondrial DNA lacking the heavy-strand promoter region". Mol. Cell. Biol. 11 (3): 1631–7. PMC 369459. PMID 1996112. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=369459. 
• Attardi G, Chomyn A, Doolittle RF, et al. (1987). "Seven unidentified reading frames of human mitochondrial DNA encode subunits of the respiratory chain NADH dehydrogenase". Cold Spring Harb. Symp. Quant. Biol. 51 Pt 1: 103–14. PMID 3472707. 
• Chomyn A, Cleeter MW, Ragan CI, et al. (1986). "URF6, last unidentified reading frame of human mtDNA, codes for an NADH dehydrogenase subunit". Science 234 (4776): 614–8. doi:10.1126/science.3764430. PMID 3764430. 
• Chomyn A, Mariottini P, Cleeter MW, et al. (1985). "Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase". Nature 314 (6012): 592–7. doi:10.1038/314592a0. PMID 3921850. 
• Sanger F, Coulson AR, Barrell BG, et al. (1981). "Cloning in single-stranded bacteriophage as an aid to rapid DNA sequencing". J. Mol. Biol. 143 (2): 161–78. doi:10.1016/0022-2836(80)90196-5. PMID 6260957. 
• Montoya J, Ojala D, Attardi G (1981). "Distinctive features of the 5'-terminal sequences of the human mitochondrial mRNAs". Nature 290 (5806): 465–70. doi:10.1038/290465a0. PMID 7219535. 
• Horai S, Hayasaka K, Kondo R, et al. (1995). "Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs". Proc. Natl. Acad. Sci. U.S.A. 92 (2): 532–6. doi:10.1073/pnas.92.2.532. PMC 42775. PMID 7530363. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=42775. 
• Gattermann N, Retzlaff S, Wang YL, et al. (1997). "Heteroplasmic point mutations of mitochondrial DNA affecting subunit I of cytochrome c oxidase in two patients with acquired idiopathic sideroblastic anemia". Blood 90 (12): 4961–72. PMID 9389715. 
• Bröker S, Meunier B, Rich P, et al. (1998). "MtDNA mutations associated with sideroblastic anaemia cause a defect of mitochondrial cytochrome c oxidase". Eur. J. Biochem. 258 (1): 132–8. doi:10.1046/j.1432-1327.1998.2580132.x. PMID 9851701. 
• Andrews RM, Kubacka I, Chinnery PF, et al. (1999). "Reanalysis and revision of the Cambridge reference sequence for human mitochondrial DNA". Nat. Genet. 23 (2): 147. doi:10.1038/13779. PMID 10508508. 
• Karadimas CL, Greenstein P, Sue CM, et al. (2000). "Recurrent myoglobinuria due to a nonsense mutation in the COX I gene of mitochondrial DNA". Neurology 55 (5): 644–9. PMID 10980727. 
• Ingman M, Kaessmann H, Pääbo S, Gyllensten U (2001). "Mitochondrial genome variation and the origin of modern humans". Nature 408 (6813): 708–13. doi:10.1038/35047064. PMID 11130070. 
• Finnilä S, Lehtonen MS, Majamaa K (2001). "Phylogenetic network for European mtDNA". Am. J. Hum. Genet. 68 (6): 1475–84. doi:10.1086/320591. PMC 1226134. PMID 11349229. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1226134. 
• Maca-Meyer N, González AM, Larruga JM, et al. (2003). "Major genomic mitochondrial lineages delineate early human expansions". BMC Genet. 2: 13. doi:10.1186/1471-2156-2-13. PMC 55343. PMID 11553319. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=55343. 

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