Cytochrome c oxidase subunit 3

Name	Cytochrome c oxidase subunit 3
Synonyms	COIII COXIII Cytochrome c oxidase polypeptide III MTCO3
Gene Name	MT-CO3
Organism	Human
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 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 integral component of membrane respiratory chain complex IV mitochondrial inner membrane
General Function	Cytochrome-c oxidase activity
Specific Function	Subunits I, II and III form the functional core of the enzyme complex.
Transmembrane Regions	15-35 42-59 81-101 127-147 159-179 197-217 239-259
GenBank Protein ID	337189
UniProtKB ID	P00414
UniProtKB Entry Name	COX3_HUMAN
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
GenBank Gene ID	J01415
GeneCard ID	None
GenAtlas ID
HGNC ID	HGNC:7422
Chromosome Location	None
Locus	None
References	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 ] 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 ] 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 ] 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 ] 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 ] 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 ] 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 ] 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 ] 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 ]

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