Cytochrome c oxidase subunit 3

Details

Name
Cytochrome c oxidase subunit 3
Synonyms
  • COIII
  • COXIII
  • Cytochrome c oxidase polypeptide III
  • MTCO3
Gene Name
MT-CO3
Organism
Humans
Amino acid sequence
>lcl|BSEQ0019500|Cytochrome c oxidase subunit 3
MTHQSHAYHMVKPSPWPLTGALSALLMTSGLAMWFHFHSMTLLMLGLLTNTLTMYQWWRD
VTRESTYQGHHTPPVQKGLRYGMILFITSEVFFFAGFFWAFYHSSLAPTPQLGGHWPPTG
ITPLNPLEVPLLNTSVLLASGVSITWAHHSLMENNRNQMIQALLITILLGLYFTLLQASE
YFESPFTISDGIYGSTFFVATGFHGLHVIIGSTFLTICFIRQLMFHFTSKHHFGFEAAAW
YWHFVDVVWLFLYVSIYWWGS
Number of residues
261
Molecular Weight
29950.6
Theoretical pI
7.34
GO Classification
Functions
cytochrome-c oxidase activity
Processes
aerobic electron transport chain / cellular metabolic process / gene expression / respiratory chain complex IV assembly / respiratory electron transport chain / small molecule metabolic process / transcription initiation from RNA polymerase II promoter
Components
integral component of membrane / mitochondrial inner membrane / respiratory chain complex IV
General Function
Cytochrome-c oxidase activity
Specific Function
Subunits I, II and III form the functional core of the enzyme complex.
Pfam Domain Function
Transmembrane Regions
15-35 42-59 81-101 127-147 159-179 197-217 239-259
Cellular Location
Mitochondrion 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
Chromosome Location
Not Available
Locus
Not Available
External Identifiers
ResourceLink
UniProtKB IDP00414
UniProtKB Entry NameCOX3_HUMAN
GenBank Protein ID337189
GenBank Gene IDJ01415
HGNC IDHGNC:7422
General 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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB02659Cholic AcidapprovedunknownDetails
DB04464N-FormylmethionineexperimentalunknownDetails