Cytochrome c oxidase subunit II

Cytochrome c oxidase subunit II
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 3VRJ​
Identifiers
Symbols	COX2 ; COII; MTCO2
External IDs	OMIM: 516040 MGI: 102503 HomoloGene: 5017 ChEMBL: 6174 GeneCards: COX2 Gene
Gene ontology Molecular function • cytochrome-c oxidase activity • copper ion binding • electron carrier activity • heme binding Cellular component • mitochondrion • mitochondrial inner membrane • integral to membrane • respiratory chain Biological process • mitochondrial electron transport, cytochrome c to oxygen • lactation • response to cold • respiratory electron transport chain • small molecule metabolic process Sources: Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	4513	17709
Ensembl	ENSG00000198712	ENSMUSG00000064354
UniProt	P00403	P00405
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	n/a	NP_904331
Location (UCSC)	Chr MT: 0.01 – 0.01 Mb	Chr MT: 0.01 – 0.01 Mb
PubMed search

Cytochrome c oxidase subunit II, transmembrane domain
Bacterial cytochrome c oxidase complex. Subunit II indicated by blue.
Identifiers
Symbol	COX2_TM
Pfam	PF02790
InterPro	IPR011759
PROSITE	PDOC00075
SCOP	1occ
SUPERFAMILY	1occ
TCDB	3.D.4
OPM superfamily	4
OPM protein	1v55
Available protein structures: Pfam structures PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

Cytochrome C oxidase subunit II, periplasmic domain
Identifiers
Symbol	COX2
Pfam	PF00116
InterPro	IPR002429
PROSITE	PDOC00075
SCOP	1occ
SUPERFAMILY	1occ
TCDB	3.D.4
OPM superfamily	4
OPM protein	1v55
CDD	cd13912
Available protein structures: Pfam structures PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

Cytochrome c oxidase subunit 2, also known as cytochrome c oxidase polypeptide II, is a protein that in humans is encoded by the MT-CO2 gene.^[1]

Cytochrome c oxidase subunit II, abbreviated COXII, COX2, COII, or MT-CO2, is the second subunit of cytochrome c oxidase.

Structure

Cytochrome c oxidase (EC 1.9.3.1)^[2][3] is an oligomeric enzymatic complex which is a component of the respiratory chain and is involved in the transfer of electrons from cytochrome c to oxygen. In eukaryotes this enzyme complex is located in the mitochondrial inner membrane; in aerobic prokaryotes it is found in the plasma membrane. The enzyme complex consists of 3-4 subunits (prokaryotes) to up to 13 polypeptides (mammals). In Leigh's disease, there may be an abnormality or deficiency of cytochrome oxidase.

Function

Subunit 2 (COII) transfers the electrons from cytochrome c to the catalytic subunit 1. It contains two adjacent transmembrane regions in its N-terminus and the major part of the protein is exposed to the periplasmic or to the mitochondrial intermembrane space, respectively. COII provides the substrate-binding site and contains a copper centre called Cu(A) (see IPR001505), probably the primary acceptor in cytochrome c oxidase. An exception is the corresponding subunit of the cbb3-type oxidase which lacks the copper A redox-centre. Several bacterial COII have a C-terminal extension that contains a covalently bound haem c.

The N-terminal domain of cytochrome C oxidase contains two transmembrane alpha-helices.

Further reading

• Torroni A, Achilli A, Macaulay V, Richards M, Bandelt HJ (Jun 2006). "Harvesting the fruit of the human mtDNA tree". Trends in Genetics 22 (6): 339–45. doi:10.1016/j.tig.2006.04.001. PMID 16678300.
• Barrell BG, Bankier AT, Drouin J (Nov 1979). "A different genetic code in human mitochondria". Nature 282 (5735): 189–94. doi:10.1038/282189a0. PMID 226894.
• Bodenteich A, Mitchell LG, Polymeropoulos MH, Merril CR (May 1992). "Dinucleotide repeat in the human mitochondrial D-loop". Human Molecular Genetics 1 (2): 140. doi:10.1093/hmg/1.2.140-a. PMID 1301157.
• Lu X, Walker T, MacManus JP, Seligy VL (Jul 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 Research 52 (13): 3718–25. PMID 1377597.
• Marzuki S, Noer AS, Lertrit P, Thyagarajan D, Kapsa R, Utthanaphol P, Byrne E (Dec 1991). "Normal variants of human mitochondrial DNA and translation products: the building of a reference data base". Human Genetics 88 (2): 139–45. doi:10.1007/bf00206061. PMID 1757091.
• Moraes CT, Andreetta F, Bonilla E, Shanske S, DiMauro S, Schon EA (Mar 1991). "Replication-competent human mitochondrial DNA lacking the heavy-strand promoter region". Molecular and Cellular Biology 11 (3): 1631–7. PMC 369459. PMID 1996112.
• Power MD, Kiefer MC, Barr PJ, Reeves R (Aug 1989). "Nucleotide sequence of human mitochondrial cytochrome c oxidase II cDNA". Nucleic Acids Research 17 (16): 6734. doi:10.1093/nar/17.16.6734. PMC 318375. PMID 2550900.
• Attardi G, Chomyn A, Doolittle RF, Mariottini P, Ragan CI (1987). "Seven unidentified reading frames of human mitochondrial DNA encode subunits of the respiratory chain NADH dehydrogenase". Cold Spring Harbor Symposia on Quantitative Biology 51 (1): 103–14. doi:10.1101/sqb.1986.051.01.013. PMID 3472707.
• Chomyn A, Cleeter MW, Ragan CI, Riley M, Doolittle RF, Attardi G (Oct 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, Ragan CI, Matsuno-Yagi A, Hatefi Y, Doolittle RF, Attardi G (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, de Bruijn MH, Coulson AR, Drouin J, Eperon IC, Nierlich DP, Roe BA, Sanger F, Schreier PH, Smith AJ, Staden R, Young IG (Apr 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 (Apr 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, Tsugane K, Takahata N (Jan 1995). "Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs". Proceedings of the National Academy of Sciences of the United States of America 92 (2): 532–6. doi:10.1073/pnas.92.2.532. PMC 42775. PMID 7530363.
• Ruvolo M, Zehr S, von Dornum M, Pan D, Chang B, Lin J (Nov 1993). "Mitochondrial COII sequences and modern human origins". Molecular Biology and Evolution 10 (6): 1115–35. PMID 8277847.
• Polyak K, Li Y, Zhu H, Lengauer C, Willson JK, Markowitz SD, Trush MA, Kinzler KW, Vogelstein B (Nov 1998). "Somatic mutations of the mitochondrial genome in human colorectal tumours". Nature Genetics 20 (3): 291–3. doi:10.1038/3108. PMID 9806551.
• Kato MV (Mar 1999). "The mechanisms of death of an erythroleukemic cell line by p53: involvement of the microtubule and mitochondria". Leukemia & Lymphoma 33 (1-2): 181–6. doi:10.3109/10428199909093740. PMID 10194136.
• Andrews RM, Kubacka I, Chinnery PF, Lightowlers RN, Turnbull DM, Howell N (Oct 1999). "Reanalysis and revision of the Cambridge reference sequence for human mitochondrial DNA". Nature Genetics 23 (2): 147. doi:10.1038/13779. PMID 10508508.
• Ingman M, Kaessmann H, Pääbo S, Gyllensten U (Dec 2000). "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 (Jun 2001). "Phylogenetic network for European mtDNA". American Journal of Human Genetics 68 (6): 1475–84. doi:10.1086/320591. PMC 1226134. PMID 11349229.
• Maca-Meyer N, González AM, Larruga JM, Flores C, Cabrera VM (2003). "Major genomic mitochondrial lineages delineate early human expansions". BMC Genetics 2: 13. doi:10.1186/1471-2156-2-13. PMC 55343. PMID 11553319.

References

1. "Entrez Gene: COX2 cytochrome c oxidase subunit II".
2. Capaldi RA, Malatesta F, Darley-Usmar VM (Jul 1983). "Structure of cytochrome c oxidase". Biochimica et Biophysica Acta 726 (2): 135–48. doi:10.1016/0304-4173(83)90003-4. PMID 6307356.
3. García-Horsman JA, Barquera B, Rumbley J, Ma J, Gennis RB (Sep 1994). "The superfamily of heme-copper respiratory oxidases". Journal of Bacteriology 176 (18): 5587–600. PMC 196760. PMID 8083153.