NameCytochrome c oxidase subunit 2
Synonyms
  • COII
  • COXII
  • Cytochrome c oxidase polypeptide II
  • MTCO2
Gene NameMT-CO2
OrganismHuman
Amino acid sequence
>lcl|BSEQ0012366|Cytochrome c oxidase subunit 2
MAHAAQVGLQDATSPIMEELITFHDHALMIIFLICFLVLYALFLTLTTKLTNTNISDAQE
METVWTILPAIILVLIALPSLRILYMTDEVNDPSLTIKSIGHQWYWTYEYTDYGGLIFNS
YMLPPLFLEPGDLRLLDVDNRVVLPIEAPIRMMITSQDVLHSWAVPTLGLKTDAIPGRLN
QTTFTATRPGVYYGQCSEICGANHSFMPIVLELIPLKIFEMGPVFTL
Number of residues227
Molecular Weight25564.73
Theoretical pI4.44
GO Classification
Functions
  • copper ion binding
  • cytochrome-c oxidase activity
Processes
  • lactation
  • gene expression
  • small molecule metabolic process
  • transcription initiation from RNA polymerase II promoter
  • cellular metabolic process
  • respiratory electron transport chain
  • hydrogen ion transmembrane transport
  • response to cold
  • mitochondrial electron transport, cytochrome c to oxygen
Components
  • mitochondrial inner membrane
  • integral component of membrane
  • extracellular exosome
  • respiratory chain complex IV
  • mitochondrion
  • membrane
General FunctionCytochrome-c oxidase activity
Specific FunctionCytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1.
Pfam Domain Function
Transmembrane Regions27-48 63-82
GenBank Protein IDNot Available
UniProtKB IDP00403
UniProtKB Entry NameCOX2_HUMAN
Cellular LocationMitochondrion inner membrane
Gene sequence
>lcl|BSEQ0012367|Cytochrome c oxidase subunit 2 (MT-CO2)
ATGGCACATGCAGCGCAAGTAGGTCTACAAGACGCTACTTCCCCTATCATAGAAGAGCTT
ATCACCTTTCATGATCACGCCCTCATAATCATTTTCCTTATCTGCTTCCTAGTCCTGTAT
GCCCTTTTCCTAACACTCACAACAAAACTAACTAATACTAACATCTCAGACGCTCAGGAA
ATAGAAACCGTCTGAACTATCCTGCCCGCCATCATCCTAGTCCTCATCGCCCTCCCATCC
CTACGCATCCTTTACATAACAGACGAGGTCAACGATCCCTCCCTTACCATCAAATCAATT
GGCCACCAATGGTACTGAACCTACGAGTACACCGACTACGGCGGACTAATCTTCAACTCC
TACATACTTCCCCCATTATTCCTAGAACCAGGCGACCTGCGACTCCTTGACGTTGACAAT
CGAGTAGTACTCCCGATTGAAGCCCCCATTCGTATAATAATTACATCACAAGACGTCTTG
CACTCATGAGCTGTCCCCACATTAGGCTTAAAAACAGATGCAATTCCCGGACGTCTAAAC
CAAACCACTTTCACCGCTACACGACCGGGGGTATACTACGGTCAATGCTCTGAAATCTGT
GGAGCAAACCACAGTTTCATGCCCATCGTCCTAGAATTAATTCCCCTAAAAATCTTTGAA
ATAGGGCCCGTATTTACCCTATAG
GenBank Gene IDV00662
GeneCard IDNot Available
GenAtlas IDMT-CO2
HGNC IDHGNC:7421
Chromosome LocationNot Available
Locus-
References
  1. 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: Sequence and organization of the human mitochondrial genome. Nature. 1981 Apr 9;290(5806):457-65. 7219534
  2. Power MD, Kiefer MC, Barr PJ, Reeves R: Nucleotide sequence of human mitochondrial cytochrome c oxidase II cDNA. Nucleic Acids Res. 1989 Aug 25;17(16):6734. 2550900
  3. Barrell BG, Bankier AT, Drouin J: A different genetic code in human mitochondria. Nature. 1979 Nov 8;282(5735):189-94. 226894
  4. Horai S, Hayasaka K, Kondo R, Tsugane K, Takahata N: Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs. Proc Natl Acad Sci U S A. 1995 Jan 17;92(2):532-6. 7530363
  5. Ruvolo M, Zehr S, von Dornum M, Pan D, Chang B, Lin J: Mitochondrial COII sequences and modern human origins. Mol Biol Evol. 1993 Nov;10(6):1115-35. 8277847
  6. Moilanen JS, Finnila S, Majamaa K: Lineage-specific selection in human mtDNA: lack of polymorphisms in a segment of MTND5 gene in haplogroup J. Mol Biol Evol. 2003 Dec;20(12):2132-42. Epub 2003 Aug 29. 12949126
  7. Ingman M, Kaessmann H, Paabo S, Gyllensten U: Mitochondrial genome variation and the origin of modern humans. Nature. 2000 Dec 7;408(6813):708-13. 11130070
  8. Ingman M, Gyllensten U: Mitochondrial genome variation and evolutionary history of Australian and New Guinean aborigines. Genome Res. 2003 Jul;13(7):1600-6. 12840039
  9. Coble MD, Just RS, O'Callaghan JE, Letmanyi IH, Peterson CT, Irwin JA, Parsons TJ: Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in Caucasians. Int J Legal Med. 2004 Jun;118(3):137-46. Epub 2004 Feb 4. 14760490
  10. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J: Initial characterization of the human central proteome. BMC Syst Biol. 2011 Jan 26;5:17. doi: 10.1186/1752-0509-5-17. 21269460
  11. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. 24275569
  12. Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. 25944712
  13. Marzuki S, Noer AS, Lertrit P, Thyagarajan D, Kapsa R, Utthanaphol P, Byrne E: Normal variants of human mitochondrial DNA and translation products: the building of a reference data base. Hum Genet. 1991 Dec;88(2):139-45. 1757091
  14. Polyak K, Li Y, Zhu H, Lengauer C, Willson JK, Markowitz SD, Trush MA, Kinzler KW, Vogelstein B: Somatic mutations of the mitochondrial genome in human colorectal tumours. Nat Genet. 1998 Nov;20(3):291-3. 9806551
  15. Rahman S, Taanman JW, Cooper JM, Nelson I, Hargreaves I, Meunier B, Hanna MG, Garcia JJ, Capaldi RA, Lake BD, Leonard JV, Schapira AH: A missense mutation of cytochrome oxidase subunit II causes defective assembly and myopathy. Am J Hum Genet. 1999 Oct;65(4):1030-9. 10486321