MT-CO3

"COX3" redirects here. For the cyclooxygenase isoenzyme, see COX-3.

Cytochrome c oxidase III
Identifiers
Symbols	COX3; COIII; MTCO3
External IDs	OMIM: 516050 MGI: 102502 HomoloGene: 5014 GeneCards: COX3 Gene
EC number	1.9.3.1
Gene Ontology Molecular function • cytochrome-c oxidase activity Cellular component • mitochondrial inner membrane • integral to membrane Biological process • mitochondrial electron transport, cytochrome c to oxygen • aerobic electron transport chain • respiratory electron transport chain • small molecule metabolic process Sources: Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	4514	17710
Ensembl	ENSG00000198938	ENSMUSG00000064358
UniProt	P00414	Q5MCM5
RefSeq (mRNA)	YP_003024032	n/a
RefSeq (protein)	YP_003024032	NP_904334
Location (UCSC)	Chr MT: 0.01 – 0.01 Mb	Chr MT: 0.01 – 0.01 Mb
PubMed search	[1]	[2]

Cytochrome c oxidase subunit 3 is an enzyme that in humans is encoded by the MT-CO3 gene.

Clinical significance

Mutations in mtDNA-encoded cytochrome c oxidase subunit genes have been observed to be associated with isolated myopathy or severe encephalomyopathy.

Further reading

• 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. 
• 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. 
• Anderson S, Bankier AT, Barrell BG, et al. (1981). "Sequence and organization of the human mitochondrial genome". Nature 290 (5806): 457–65. doi:10.1038/290457a0. PMID 7219534. 
• 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. 
• 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. 
• 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. 
• 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. 
• Herrnstadt C, Elson JL, Fahy E, et al. (2002). "Reduced-Median-Network Analysis of Complete Mitochondrial DNA Coding-Region Sequences for the Major African, Asian, and European Haplogroups". Am. J. Hum. Genet. 70 (5): 1152–71. doi:10.1086/339933. PMC 447592. PMID 11938495. 
• Silva WA, Bonatto SL, Holanda AJ, et al. (2002). "Mitochondrial Genome Diversity of Native Americans Supports a Single Early Entry of Founder Populations into America". Am. J. Hum. Genet. 71 (1): 187–92. doi:10.1086/341358. PMC 384978. PMID 12022039. 
• Elkon H, Don J, Melamed E, et al. (2003). "Mutant and wild-type alpha-synuclein interact with mitochondrial cytochrome C oxidase". J. Mol. Neurosci. 18 (3): 229–38. doi:10.1385/JMN:18:3:229. PMID 12059041. 
• Mishmar D, Ruiz-Pesini E, Golik P, et al. (2003). "Natural selection shaped regional mtDNA variation in humans". Proc. Natl. Acad. Sci. U.S.A. 100 (1): 171–6. doi:10.1073/pnas.0136972100. PMC 140917. PMID 12509511. 
• Ingman M, Gyllensten U (2003). "Mitochondrial Genome Variation and Evolutionary History of Australian and New Guinean Aborigines". Genome Res. 13 (7): 1600–6. doi:10.1101/gr.686603. PMC 403733. PMID 12840039. 
• Kong QP, Yao YG, Sun C, et al. (2003). "Phylogeny of East Asian Mitochondrial DNA Lineages Inferred from Complete Sequences". Am. J. Hum. Genet. 73 (3): 671–6. doi:10.1086/377718. PMC 1180693. PMID 12870132. 
• Temperley RJ, Seneca SH, Tonska K, et al. (2004). "Investigation of a pathogenic mtDNA microdeletion reveals a translation-dependent deadenylation decay pathway in human mitochondria". Hum. Mol. Genet. 12 (18): 2341–8. doi:10.1093/hmg/ddg238. PMID 12915481. 
• Maca-Meyer N, González AM, Pestano J, et al. (2004). "Mitochondrial DNA transit between West Asia and North Africa inferred from U6 phylogeography". BMC Genet. 4: 15. doi:10.1186/1471-2156-4-15. PMC 270091. PMID 14563219. 
• Coble MD, Just RS, O'Callaghan JE, et al. (2004). "Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in Caucasians". Int. J. Legal Med. 118 (3): 137–46. doi:10.1007/s00414-004-0427-6. PMID 14760490. 
• Palanichamy MG, Sun C, Agrawal S, et al. (2005). "Phylogeny of Mitochondrial DNA Macrohaplogroup N in India, Based on Complete Sequencing: Implications for the Peopling of South Asia". Am. J. Hum. Genet. 75 (6): 966–78. doi:10.1086/425871. PMC 1182158. PMID 15467980. 
• Starikovskaya EB, Sukernik RI, Derbeneva OA, et al. (2005). "Mitochondrial DNA diversity in indigenous populations of the southern extent of Siberia, and the origins of Native American haplogroups". Ann. Hum. Genet. 69 (Pt 1): 67–89. doi:10.1046/j.1529-8817.2003.00127.x. PMID 15638829. 
• Rajkumar R, Banerjee J, Gunturi HB, et al. (2006). "Phylogeny and antiquity of M macrohaplogroup inferred from complete mt DNA sequence of Indian specific lineages". BMC Evol. Biol. 5: 26. doi:10.1186/1471-2148-5-26. PMC 1079809. PMID 15804362. 

External links

• GeneReviews/NCBI/NIH/UW entry on Mitochondrial DNA-Associated Leigh Syndrome and NARP