MT-CO1

Cytochrome c oxidase subunit I
	; PDB rendering based on 1occ.
Available structures
PDB	Ortholog search: PDBe, RCSB
List of PDB id codes
	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 ChEMBL: 6173 GeneCards: COX1 Gene
Gene Ontology
Molecular function	• cytochrome-c oxidase activity; • iron ion binding; • protein binding; • electron carrier activity; • heme binding;
Cellular component	• mitochondrial inner membrane; • integral to membrane; • respiratory chain;
Biological process	• mitochondrial electron transport, cytochrome c to oxygen; • response to oxidative stress; • aging; • aerobic respiration; • cerebellum development; • respiratory electron transport chain; • small molecule metabolic process; • response to copper ion; • response to electrical stimulus;
	Sources: Amigo / QuickGO
Orthologs
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"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 or COI.^[1]

Function[edit]

MT-CO1 belongs to cytochrome c oxidase subunit I protein family. Cytochrome c oxidase subunit I (CO1 or MT-CO1) is one of three mitochondrial DNA (mtDNA) encoded subunits (MT-CO1, MT-CO2, MT-CO3) of respiratory complex IV. Complex IV is the third and final enzyme of the electron transport chain of mitochondrial oxidative phosphorylation.^[2]

Application[edit]

It is a gene that is often used as "barcode of life" in DNA barcoding to identify species. MT-CO1 gene sequence is suitable for this role because its mutation rate is often fast enough to distinguish closely related species and also because its sequence is conserved among conspecifics. Contrary to the primary objection raised by skeptics that MT-CO1 sequence differences are too small to be detected between closely related species, more than 2% sequence divergence has been detected between such organisms,^[3] proving the barcode effective. In most if not all seed plants, however, the rate of evolution of cox1 is very slow.

References[edit]

^ 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". Protist 163 (3): 415–34. doi:10.1016/j.protis.2011.10.003. PMID 22130576.
^ "Entrez Gene: COX1 cytochrome c oxidase subunit I".
^ Hebert PD, Ratnasingham S, deWaard JR (August 2003). "Barcoding animal life: cytochrome c oxidase subunit 1 divergences among closely related species". Proc. Biol. Sci. 270 Suppl 1: S96–9. doi:10.1098/rsbl.2003.0025. PMC 1698023. PMID 12952648.

Further reading[edit]

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.
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.
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.
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.
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.