ID Q6VHV2_HUMAN Unreviewed; 513 AA. AC Q6VHV2; DT 05-JUL-2004, integrated into UniProtKB/TrEMBL. DT 05-JUL-2004, sequence version 1. DT 19-MAR-2014, entry version 80. DE RecName: Full=Cytochrome c oxidase subunit 1; DE EC=1.9.3.1; GN Name=COX1; OS Homo sapiens (Human). OG Mitochondrion. OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; OC Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; OC Catarrhini; Hominidae; Homo. OX NCBI_TaxID=9606; RN [1] RP NUCLEOTIDE SEQUENCE. RC STRAIN=F164; RX PubMed=11349229; DOI=10.1086/320591; RA Finnila S., Lehtonen M.S., Majamaa K.; RT "Phylogenetic network for European mtDNA."; RL Am. J. Hum. Genet. 68:1475-1484(2001). RN [2] RP NUCLEOTIDE SEQUENCE. RC STRAIN=MTDNA361; RX PubMed=11938495; DOI=10.1086/339933; RA Herrnstadt C., Elson J.L., Fahy E., Preston G., Turnbull D.M., RA Anderson C., Ghosh S.S., Olefsky J.M., Beal M.F., Davis R.E., RA Howell N.; RT "Reduced-median-network analysis of complete mitochondrial DNA coding- RT region sequences for the major African, Asian, and European RT haplogroups."; RL Am. J. Hum. Genet. 70:1152-1171(2002). RN [3] RP NUCLEOTIDE SEQUENCE. RC STRAIN=F164; RX PubMed=12949126; DOI=10.1093/molbev/msg230; RA Moilanen J.S., Finnila S., Majamaa K.; RT "Lineage-specific selection in human mtDNA: lack of polymorphisms in a RT segment of MTND5 gene in haplogroup J."; RL Mol. Biol. Evol. 20:2132-2142(2003). RN [4] RP NUCLEOTIDE SEQUENCE. RC STRAIN=39_M1a5; RX PubMed=17170302; DOI=10.1126/science.1135566; RA Olivieri A., Achilli A., pala M., Battaglia V., Fornarino S., RA Al-Zahery N., Scozzari R., Cruciani F., Behar D.M., Dugoujon J.-M., RA Coudray C., Santachiara-Benerecetti A.S., Semino O., Bandelt H.-J., RA Torroni A.; RT "The mtDNA legacy of the Levantine early Upper Palaeolithic in RT Africa."; RL Science 314:1767-1770(2006). RN [5] RP NUCLEOTIDE SEQUENCE. RC STRAIN=F164; RA Moilanen J.S., Finnila S., Lehtonen M.S., Majamaa K.; RL Submitted (JUL-2006) to the EMBL/GenBank/DDBJ databases. RN [6] RP NUCLEOTIDE SEQUENCE. RC STRAIN=MTDNA361; RG HmtDB; RL Submitted (JUN-2007) to the EMBL/GenBank/DDBJ databases. RN [7] RP NUCLEOTIDE SEQUENCE. RC STRAIN=MTDNA361; RA Herrnstadt C., Fahy E., Preston G., Anderson C., Ghosh S.S., RA Davis R.E., Howell N.; RL Submitted (JUN-2007) to the EMBL/GenBank/DDBJ databases. RN [8] RP NUCLEOTIDE SEQUENCE. RC STRAIN=Am012, and AM038; RX PubMed=18359946; DOI=10.1093/molbev/msn068; RA Soares P., Trejaut J.A., Loo J.H., Hill C., Mormina M., Lee C.L., RA Chen Y.M., Hudjashov G., Forster P., Macaulay V., Bulbeck D., RA Oppenheimer S., Lin M., Richards M.B.; RT "Climate change and postglacial human dispersals in southeast Asia."; RL Mol. Biol. Evol. 25:1209-1218(2008). RN [9] RP NUCLEOTIDE SEQUENCE. RC STRAIN=KD10, KD20, KD23, KD27, and KR30; RX PubMed=19624810; DOI=10.1186/1471-2148-9-173; RA Kumar S., Ravuri R.R., Koneru P., Urade B.P., Sarkar B.N., RA Chandrasekar A., Rao V.R.; RT "Reconstructing Indian-Australian phylogenetic link."; RL BMC Evol. Biol. 9:173-173(2009). RN [10] RP NUCLEOTIDE SEQUENCE. RC STRAIN=KD10, KD20, KD23, KD27, and KR30; RX PubMed=19823670; DOI=10.1371/journal.pone.0007447; RA Chandrasekar A., Kumar S., Sreenath J., Sarkar B.N., Urade B.P., RA Mallick S., Bandopadhyay S.S., Barua P., Barik S.S., Basu D., RA Kiran U., Gangopadhyay P., Sahani R., Prasad B.V., Gangopadhyay S., RA Lakshmi G.R., Ravuri R.R., Padmaja K., Venugopal P.N., Sharma M.B., RA Rao V.R.; RT "Updating phylogeny of mitochondrial DNA macrohaplogroup m in India: RT dispersal of modern human in South Asian corridor."; RL PLoS ONE 4:E7447-E7447(2009). RN [11] RP NUCLEOTIDE SEQUENCE. RX PubMed=21203537; DOI=10.1371/journal.pone.0015214; RA Derenko M., Malyarchuk B., Grzybowski T., Denisova G., Rogalla U., RA Perkova M., Dambueva I., Zakharov I.; RT "Origin and post-glacial dispersal of mitochondrial DNA haplogroups C RT and D in northern Asia."; RL PLoS ONE 5:E15214-E15214(2010). RN [12] RP NUCLEOTIDE SEQUENCE. RA Sharma V., Singh L., Thangaraj K., Nandan A., Sharma V.K.; RT "Phylogenomic study of a concealed Ladakh tribe of the Great RT Himalayas."; RL Submitted (MAR-2010) to the EMBL/GenBank/DDBJ databases. RN [13] RP NUCLEOTIDE SEQUENCE. RA Greenspan B.; RL Submitted (JUL-2010) to the EMBL/GenBank/DDBJ databases. RN [14] RP NUCLEOTIDE SEQUENCE. RX PubMed=21978175; DOI=10.1186/1471-2148-11-293; RA Kumar S., Bellis C., Zlojutro M., Melton P.E., Blangero J., RA Curran J.E.; RT "Large scale mitochondrial sequencing in Mexican Americans suggests a RT reappraisal of Native American origins."; RL BMC Evol. Biol. 11:293-293(2011). RN [15] RP NUCLEOTIDE SEQUENCE. RA Schoenberg A., Theunert C., Li M., Stoneking M., Nasidze I.; RT "High-throughput sequencing of complete human mtDNA genomes from the RT Caucasus and West Asia: high diversity and demographic inferences."; RL Eur. J. Hum. Genet. 0:0-0(2011). RN [16] RP NUCLEOTIDE SEQUENCE. RA Gomez-Carballa A., Olivieri A., Behar D., Achilli A., Torroni A.; RT "Genetic continuity in the Franco-Cantabrian region: new clues from a RT mtDNA lineage."; RL Submitted (JUL-2011) to the EMBL/GenBank/DDBJ databases. RN [17] RP NUCLEOTIDE SEQUENCE. RX PubMed=22482806; DOI=10.1016/j.ajhg.2012.03.002; RA Behar D.M., van Oven M., Rosset S., Metspalu M., Loogvali E.L., RA Silva N.M., Kivisild T., Torroni A., Villems R.; RT "A "Copernican" reassessment of the human mitochondrial DNA tree from RT its root."; RL Am. J. Hum. Genet. 90:675-684(2012). RN [18] RP NUCLEOTIDE SEQUENCE. RX PubMed=22560092; DOI=10.1016/j.ajhg.2012.04.003; RA Pala M., Olivieri A., Achilli A., Accetturo M., Metspalu E., RA Reidla M., Tamm E., Karmin M., Reisberg T., Hooshiar Kashani B., RA Perego U.A., Carossa V., Gandini F., Pereira J.B., Soares P., RA Angerhofer N., Rychkov S., Al-Zahery N., Carelli V., Sanati M.H., RA Houshmand M., Hatina J., Macaulay V., Pereira L., Woodward S.R., RA Davies W., Gamble C., Baird D., Semino O., Villems R., Torroni A., RA Richards M.B.; RT "Mitochondrial DNA signals of late glacial recolonization of europe RT from near eastern refugia."; RL Am. J. Hum. Genet. 90:915-924(2012). RN [19] RP NUCLEOTIDE SEQUENCE. RX PubMed=22363811; DOI=10.1371/journal.pone.0032179; RA Derenko M., Malyarchuk B., Denisova G., Perkova M., Rogalla U., RA Grzybowski T., Khusnutdinova E., Dambueva I., Zakharov I.; RT "Complete mitochondrial DNA analysis of eastern eurasian haplogroups RT rarely found in populations of northern Asia and eastern europe."; RL PLoS ONE 7:E32179-E32179(2012). RN [20] RP NUCLEOTIDE SEQUENCE. RC TISSUE=Saliva; RA Tito R.Y., Polo S.I., Lewis C.M.Jr.; RT "Mitochondrial Genomes and Founder Effects within the Americas."; RL Submitted (SEP-2012) to the EMBL/GenBank/DDBJ databases. RN [21] RP NUCLEOTIDE SEQUENCE. RX PubMed=24341918; RA Raule N., Sevini F., Li S., Barbieri A., Tallaro F., Lomartire L., RA Vianello D., Montesanto A., Moilanen J.S., Bezrukov V., Blanche H., RA Hervonen A., Christensen K., Deiana L., Gonos E.S., Kirkwood T.B., RA Kristensen P., Leon A., Pelicci P.G., Poulain M., Rea I.M., RA Remacle J., Robine J.M., Schreiber S., Sikora E., Eline Slagboom P., RA Spazzafumo L., Antonietta Stazi M., Toussaint O., Vaupel J.W., RA Rose G., Majamaa K., Perola M., Johnson T.E., Bolund L., Yang H., RA Passarino G., Franceschi C.; RT "The co-occurrence of mtDNA mutations on different oxidative RT phosphorylation subunits, not detected by haplogroup analysis, affects RT human longevity and is population specific."; RL Aging Cell 0:0-0(2013). RN [22] RP NUCLEOTIDE SEQUENCE. RX PubMed=23527064; RA Taboada-Echalar P., Alvarez-Iglesias V., Heinz T., Vidal-Bralo L., RA Gomez-Carballa A., Catelli L., Pardo-Seco J., Pastoriza A., RA Carracedo A., Torres-Balanza A., Rocabado O., Vullo C., Salas A.; RT "The genetic legacy of the pre-colonial period in contemporary RT bolivians."; RL PLoS ONE 8:E58980-E58980(2013). RN [23] RP NUCLEOTIDE SEQUENCE. RA Greenspan B.; RL Submitted (APR-2013) to the EMBL/GenBank/DDBJ databases. RN [24] RP NUCLEOTIDE SEQUENCE. RA Greenspan B.; RL Submitted (MAY-2013) to the EMBL/GenBank/DDBJ databases. RN [25] RP NUCLEOTIDE SEQUENCE. RC TISSUE=Peripheral blood; RA Jiang C., Cui J., Liu F., Gao L., Luo Y., Li P., Guan L., Gao Y.; RT "Mitochondrial DNA 10609T promotes hypoxia-induced increase of RT intracellular ROS and is a risk factor of high altitude RT polycythemia."; RL Submitted (NOV-2013) to the EMBL/GenBank/DDBJ databases. CC -!- FUNCTION: Cytochrome c oxidase is the component of the respiratory CC chain that catalyzes the reduction of oxygen to water. Subunits 1- CC 3 form the functional core of the enzyme complex. CO I is the CC catalytic subunit of the enzyme. Electrons originating in CC cytochrome c are transferred via the copper A center of subunit 2 CC and heme A of subunit 1 to the bimetallic center formed by heme A3 CC and copper B (By similarity). CC -!- CATALYTIC ACTIVITY: 4 ferrocytochrome c + O(2) + 4 H(+) = 4 CC ferricytochrome c + 2 H(2)O. CC -!- PATHWAY: Energy metabolism; oxidative phosphorylation. CC -!- SUBCELLULAR LOCATION: Mitochondrion inner membrane; Multi-pass CC membrane protein (By similarity). CC -!- SIMILARITY: Belongs to the heme-copper respiratory oxidase family. CC --------------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution (CC BY 4.0) License CC --------------------------------------------------------------------------- DR EMBL; AY339565; AAP91157.1; -; Genomic_DNA. DR EMBL; EF060350; ABJ89449.1; -; Genomic_DNA. DR EMBL; EF093535; ABL61582.1; -; Genomic_DNA. DR EMBL; EF093536; ABL61595.1; -; Genomic_DNA. DR EMBL; EF657522; ABR96749.1; -; Genomic_DNA. DR EMBL; FJ383536; ACM74096.1; -; Genomic_DNA. DR EMBL; FJ383537; ACM74109.1; -; Genomic_DNA. DR EMBL; FJ383538; ACM74122.1; -; Genomic_DNA. DR EMBL; FJ383539; ACM74135.1; -; Genomic_DNA. DR EMBL; FJ383540; ACM74148.1; -; Genomic_DNA. DR EMBL; FJ951544; ACQ75285.1; -; Genomic_DNA. DR EMBL; HM775494; ADK47863.1; -; Genomic_DNA. DR EMBL; HM036559; ADK73027.1; -; Genomic_DNA. DR EMBL; HM852882; ADL31534.1; -; Genomic_DNA. DR EMBL; HQ012241; ADM61076.1; -; Genomic_DNA. DR EMBL; JN214423; AEP23517.1; -; Genomic_DNA. DR EMBL; JN857039; AET09349.1; -; Genomic_DNA. DR EMBL; JQ704452; AFF95836.1; -; Genomic_DNA. DR EMBL; JQ705067; AFG03831.1; -; Genomic_DNA. DR EMBL; JQ798051; AFI53413.1; -; Genomic_DNA. DR EMBL; JX153285; AFO26880.1; -; Genomic_DNA. DR EMBL; JX669176; AFV54003.1; -; Genomic_DNA. DR EMBL; KC503926; AGH18584.1; -; Genomic_DNA. DR EMBL; KC964603; AGK41192.1; -; Genomic_DNA. DR EMBL; KF171123; AGN53292.1; -; Genomic_DNA. DR EMBL; KF849972; AHE79526.1; -; Genomic_DNA. DR HOVERGEN; HBG003841; -. DR UniPathway; UPA00705; -. DR NextBio; 35540434; -. DR GO; GO:0016021; C:integral component of membrane; IEA:UniProtKB-KW. DR GO; GO:0005743; C:mitochondrial inner membrane; IEA:UniProtKB-SubCell. DR GO; GO:0070469; C:respiratory chain; IEA:UniProtKB-KW. DR GO; GO:0004129; F:cytochrome-c oxidase activity; IEA:UniProtKB-EC. DR GO; GO:0009055; F:electron carrier activity; IEA:InterPro. DR GO; GO:0020037; F:heme binding; IEA:InterPro. DR GO; GO:0005506; F:iron ion binding; IEA:InterPro. DR GO; GO:0009060; P:aerobic respiration; IEA:InterPro. DR GO; GO:1902600; P:hydrogen ion transmembrane transport; IEA:GOC. DR GO; GO:0006119; P:oxidative phosphorylation; IEA:UniProtKB-UniPathway. DR Gene3D; 1.20.210.10; -; 1. DR InterPro; IPR000883; Cyt_c_Oxase_su1. DR InterPro; IPR023615; Cyt_c_Oxase_su1_BS. DR InterPro; IPR023616; Cyt_c_Oxase_su1_dom. DR PANTHER; PTHR10422; PTHR10422; 1. DR Pfam; PF00115; COX1; 1. DR PRINTS; PR01165; CYCOXIDASEI. DR SUPFAM; SSF81442; SSF81442; 1. DR PROSITE; PS50855; COX1; 1. DR PROSITE; PS00077; COX1_CUB; 1. PE 3: Inferred from homology; KW Copper; Electron transport; Heme; Iron; Membrane; Metal-binding; KW Mitochondrion; Mitochondrion inner membrane; Oxidoreductase; KW Respiratory chain; Transmembrane; Transport. SQ SEQUENCE 513 AA; 57053 MW; D08B485E89A3A1F1 CRC64; MFADRWLFST NHKDIGTLYL LFGAWAGVLG TALSLLIRAE LGQPGNLLGN DHIYNVIVTA HAFVMIFFMV MPIMIGGFGN WLVPLMIGAP DMAFPRMNNM SFWLLPPSLL LLLASAMVEA GAGTGWTVYP PLAGNYSHPG ASVDLIIFSL HLAGVSSILG AINFITTIIN MKPPAMTQYQ TPLFVWSVLI TAVLLLLSLP VLAAGITMLL TDRNLNTTFF DPAGGGDPIL YQHLFWFFGH PEVYILILPG FGMISHIVTY YSGKKEPFGY MGMVWAMMSI GFLGFIVWAH HMFTVGMDVD TRAYFTSATM IIAIPTGVKV FSWLATLHGS NMKWSAAVLW ALGFIFLFTV GGLTGIVLAN SSLDIVLHDT YYVVAHFHYV LSMGAVFAIM GGFIHWFPLF SGYTLDQTYA KIHFTIMFIG VNLTFFPQHF LGLSGMPRRY SDYPDAYTTW NILSSVGSFI SLTAVMLMIF MIWEAFASKR KVLMVEEPSM NLEWLYGCPP PYHTFEEPVY MKS //