Tmic
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NameCytochrome c oxidase subunit 3
Synonyms
  • COIII
  • COXIII
  • Cytochrome c oxidase polypeptide III
  • MTCO3
Gene NameMT-CO3
OrganismHuman
Amino acid sequence
>lcl|BSEQ0019500|Cytochrome c oxidase subunit 3
MTHQSHAYHMVKPSPWPLTGALSALLMTSGLAMWFHFHSMTLLMLGLLTNTLTMYQWWRD
VTRESTYQGHHTPPVQKGLRYGMILFITSEVFFFAGFFWAFYHSSLAPTPQLGGHWPPTG
ITPLNPLEVPLLNTSVLLASGVSITWAHHSLMENNRNQMIQALLITILLGLYFTLLQASE
YFESPFTISDGIYGSTFFVATGFHGLHVIIGSTFLTICFIRQLMFHFTSKHHFGFEAAAW
YWHFVDVVWLFLYVSIYWWGS
Number of residues261
Molecular Weight29950.6
Theoretical pI7.34
GO Classification
Functions
  • cytochrome-c oxidase activity
Processes
  • gene expression
  • small molecule metabolic process
  • transcription initiation from RNA polymerase II promoter
  • cellular metabolic process
  • respiratory electron transport chain
  • respiratory chain complex IV assembly
  • aerobic electron transport chain
Components
  • respiratory chain complex IV
  • mitochondrial inner membrane
  • integral component of membrane
General FunctionCytochrome-c oxidase activity
Specific FunctionSubunits I, II and III form the functional core of the enzyme complex.
Pfam Domain Function
Transmembrane Regions15-35 42-59 81-101 127-147 159-179 197-217 239-259
GenBank Protein ID337189
UniProtKB IDP00414
UniProtKB Entry NameCOX3_HUMAN
Cellular LocationMitochondrion inner membrane
Gene sequence
>lcl|BSEQ0019501|Cytochrome c oxidase subunit 3 (MT-CO3)
ATGACCCACCAATCACATGCCTATCATATAGTAAAACCCAGCCCATGACCCCTAACAGGG
GCCCTCTCAGCCCTCCTAATGACCTCCGGCCTAGCCATGTGATTTCACTTCCACTCCATA
ACGCTCCTCATACTAGGCCTACTAACCAACACACTAACCATATACCAATGATGGCGCGAT
GTAACACGAGAAAGCACATACCAAGGCCACCACACACCACCTGTCCAAAAAGGCCTTCGA
TACGGGATAATCCTATTTATTACCTCAGAAGTTTTTTTCTTCGCAGGATTTTTCTGAGCC
TTTTACCACTCCAGCCTAGCCCCTACCCCCCAATTAGGAGGGCACTGGCCCCCAACAGGC
ATCACCCCGCTAAATCCCCTAGAAGTCCCACTCCTAAACACATCCGTATTACTCGCATCA
GGAGTATCAATCACCTGAGCTCACCATAGTCTAATAGAAAACAACCGAAACCAAATAATT
CAAGCACTGCTTATTACAATTTTACTGGGTCTCTATTTTACCCTCCTACAAGCCTCAGAG
TACTTCGAGTCTCCCTTCACCATTTCCGACGGCATCTACGGCTCAACATTTTTTGTAGCC
ACAGGCTTCCACGGACTTCACGTCATTATTGGCTCAACTTTCCTCACTATCTGCTTCATC
CGCCAACTAATATTTCACTTTACATCCAAACATCACTTTGGCTTCGAAGCCGCCGCCTGA
TACTGGCATTTTGTAGATGTGGTTTGACTATTTCTGTATGTCTCCATCTATTGATGAGGG
TCTT
GenBank Gene IDJ01415
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:7422
Chromosome LocationNot Available
LocusNot Available
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. Thangaraj K, Chaubey G, Singh VK, Vanniarajan A, Thanseem I, Reddy AG, Singh L: In situ origin of deep rooting lineages of mitochondrial Macrohaplogroup 'M' in India. BMC Genomics. 2006 Jun 15;7:151. 16776823
  3. Choi BO, Hwang JH, Kim J, Cho EM, Cho SY, Hwang SJ, Lee HW, Kim SJ, Chung KW: A MELAS syndrome family harboring two mutations in mitochondrial genome. Exp Mol Med. 2008 Jun 30;40(3):354-60. 18587274
  4. 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
  5. 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
  6. Johns DR, Neufeld MJ: Cytochrome c oxidase mutations in Leber hereditary optic neuropathy. Biochem Biophys Res Commun. 1993 Oct 29;196(2):810-5. 8240356
  7. Manfredi G, Schon EA, Moraes CT, Bonilla E, Berry GT, Sladky JT, DiMauro S: A new mutation associated with MELAS is located in a mitochondrial DNA polypeptide-coding gene. Neuromuscul Disord. 1995 Sep;5(5):391-8. 7496173
  8. Keightley JA, Hoffbuhr KC, Burton MD, Salas VM, Johnston WS, Penn AM, Buist NR, Kennaway NG: A microdeletion in cytochrome c oxidase (COX) subunit III associated with COX deficiency and recurrent myoglobinuria. Nat Genet. 1996 Apr;12(4):410-6. 8630495
  9. Rieder MJ, Taylor SL, Tobe VO, Nickerson DA: Automating the identification of DNA variations using quality-based fluorescence re-sequencing: analysis of the human mitochondrial genome. Nucleic Acids Res. 1998 Feb 15;26(4):967-73. 9461455