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Identification
HMDB Protein ID HMDBP07034
Secondary Accession Numbers
  • 12653
Name Cytochrome c oxidase subunit 3
Synonyms
  1. Cytochrome c oxidase polypeptide III
Gene Name MT-CO3
Protein Type Enzyme
Biological Properties
General Function Involved in cytochrome-c oxidase activity
Specific Function Subunits I, II and III form the functional core of the enzyme complex
Pathways Not Available
Reactions Not Available
GO Classification
Component
membrane
cell part
Function
catalytic activity
oxidoreductase activity
heme-copper terminal oxidase activity
cytochrome-c oxidase activity
Process
mitochondrial electron transport, cytochrome c to oxygen
metabolic process
generation of precursor metabolites and energy
electron transport chain
respiratory electron transport chain
cellular metabolic process
Cellular Location
  1. Mitochondrion inner membrane
  2. Multi-pass membrane protein
Gene Properties
Chromosome Location Not Available
Locus Not Available
SNPs MT-CO3
Gene Sequence
>784 bp
ATGACCCACCAATCACATGCCTATCATATAGTAAAACCCAGCCCATGACCCCTAACAGGG
GCCCTCTCAGCCCTCCTAATGACCTCCGGCCTAGCCATGTGATTTCACTTCCACTCCATA
ACGCTCCTCATACTAGGCCTACTAACCAACACACTAACCATATACCAATGATGGCGCGAT
GTAACACGAGAAAGCACATACCAAGGCCACCACACACCACCTGTCCAAAAAGGCCTTCGA
TACGGGATAATCCTATTTATTACCTCAGAAGTTTTTTTCTTCGCAGGATTTTTCTGAGCC
TTTTACCACTCCAGCCTAGCCCCTACCCCCCAATTAGGAGGGCACTGGCCCCCAACAGGC
ATCACCCCGCTAAATCCCCTAGAAGTCCCACTCCTAAACACATCCGTATTACTCGCATCA
GGAGTATCAATCACCTGAGCTCACCATAGTCTAATAGAAAACAACCGAAACCAAATAATT
CAAGCACTGCTTATTACAATTTTACTGGGTCTCTATTTTACCCTCCTACAAGCCTCAGAG
TACTTCGAGTCTCCCTTCACCATTTCCGACGGCATCTACGGCTCAACATTTTTTGTAGCC
ACAGGCTTCCACGGACTTCACGTCATTATTGGCTCAACTTTCCTCACTATCTGCTTCATC
CGCCAACTAATATTTCACTTTACATCCAAACATCACTTTGGCTTCGAAGCCGCCGCCTGA
TACTGGCATTTTGTAGATGTGGTTTGACTATTTCTGTATGTCTCCATCTATTGATGAGGG
TCTT
Protein Properties
Number of Residues 261
Molecular Weight 29950.6
Theoretical pI 7.34
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • 15-35
  • 42-59
  • 81-101
  • 127-147
  • 159-179
  • 197-217
  • 239-259
Protein Sequence
>Cytochrome c oxidase subunit 3
MTHQSHAYHMVKPSPWPLTGALSALLMTSGLAMWFHFHSMTLLMLGLLTNTLTMYQWWRD
VTRESTYQGHHTPPVQKGLRYGMILFITSEVFFFAGFFWAFYHSSLAPTPQLGGHWPPTG
ITPLNPLEVPLLNTSVLLASGVSITWAHHSLMENNRNQMIQALLITILLGLYFTLLQASE
YFESPFTISDGIYGSTFFVATGFHGLHVIIGSTFLTICFIRQLMFHFTSKHHFGFEAAAW
YWHFVDVVWLFLYVSIYWWGS
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID P00414
UniProtKB/Swiss-Prot Entry Name COX3_HUMAN
PDB IDs Not Available
GenBank Gene ID J01415
GeneCard ID MT-CO3
GenAtlas ID MT-CO3
HGNC ID HGNC:7422
References
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. [PubMed:7219534 ]
  2. 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. [PubMed:1757091 ]
  3. 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. [PubMed:9461455 ]
  4. 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. [PubMed:16776823 ]
  5. Johns DR, Neufeld MJ: Cytochrome c oxidase mutations in Leber hereditary optic neuropathy. Biochem Biophys Res Commun. 1993 Oct 29;196(2):810-5. [PubMed:8240356 ]
  6. 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. [PubMed:7496173 ]
  7. 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. [PubMed:8630495 ]