ID Q6VHV2_HUMAN Unreviewed; 513 AA. AC Q6VHV2; DT 05-JUL-2004, integrated into UniProtKB/TrEMBL. DT 05-JUL-2004, sequence version 1. DT 13-SEP-2023, entry version 151. DE RecName: Full=Cytochrome c oxidase subunit 1 {ECO:0000256|ARBA:ARBA00015947, ECO:0000256|RuleBase:RU000369}; DE EC=7.1.1.9 {ECO:0000256|ARBA:ARBA00012949, ECO:0000256|RuleBase:RU000369}; GN Name=COX1 {ECO:0000313|EMBL:AAP91157.1}; GN Synonyms=COI {ECO:0000313|EMBL:QRC37185.1}, MTCO1 GN {ECO:0000313|EMBL:QRC37185.1}; OS Homo sapiens (Human). OG Mitochondrion {ECO:0000313|EMBL:AAP91157.1}. OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; OC Homo. OX NCBI_TaxID=9606 {ECO:0000313|EMBL:AAP91157.1}; RN [1] {ECO:0000313|EMBL:AAP91157.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=F164 {ECO:0000313|EMBL:AAP91157.1}; 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] {ECO:0000313|EMBL:ABR96749.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=MTDNA361 {ECO:0000313|EMBL:ABR96749.1}; RX PubMed=11938495; DOI=10.1086/339933; RA Herrnstadt C., Elson J.L., Fahy E., Preston G., Turnbull D.M., Anderson C., RA Ghosh S.S., Olefsky J.M., Beal M.F., Davis R.E., Howell N.; RT "Reduced-median-network analysis of complete mitochondrial DNA coding- RT region sequences for the major African, Asian, and European haplogroups."; RL Am. J. Hum. Genet. 70:1152-1171(2002). RN [3] {ECO:0000313|EMBL:AAP91157.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=F164 {ECO:0000313|EMBL:AAP91157.1}; 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] {ECO:0000313|EMBL:ABJ89449.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=39_M1a5 {ECO:0000313|EMBL:ABJ89449.1}; RX PubMed=17170302; DOI=10.1126/science.1135566; RA Olivieri A., Achilli A., pala M., Battaglia V., Fornarino S., Al-Zahery N., RA Scozzari R., Cruciani F., Behar D.M., Dugoujon J.-M., Coudray C., RA Santachiara-Benerecetti A.S., Semino O., Bandelt H.-J., Torroni A.; RT "The mtDNA legacy of the Levantine early Upper Palaeolithic in Africa."; RL Science 314:1767-1770(2006). RN [5] {ECO:0000313|EMBL:AAP91157.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=F164 {ECO:0000313|EMBL:AAP91157.1}; RA Moilanen J.S., Finnila S., Lehtonen M.S., Majamaa K.; RL Submitted (JUL-2006) to the EMBL/GenBank/DDBJ databases. RN [6] {ECO:0000313|EMBL:ABR96749.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=MTDNA361 {ECO:0000313|EMBL:ABR96749.1}; RA Herrnstadt C., Fahy E., Preston G., Anderson C., Ghosh S.S., Davis R.E., RA Howell N.; RL Submitted (JUN-2007) to the EMBL/GenBank/DDBJ databases. RN [7] {ECO:0000313|EMBL:ABR96749.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=MTDNA361 {ECO:0000313|EMBL:ABR96749.1}; RG HmtDB; RL Submitted (JUN-2007) to the EMBL/GenBank/DDBJ databases. RN [8] {ECO:0000313|EMBL:ABL61582.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=Am012 {ECO:0000313|EMBL:ABL61582.1}, and AM038 RC {ECO:0000313|EMBL:ABL61595.1}; 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] {ECO:0000313|EMBL:ACM74096.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=KD10 {ECO:0000313|EMBL:ACM74122.1}, KD20 RC {ECO:0000313|EMBL:ACM74096.1}, KD23 {ECO:0000313|EMBL:ACM74135.1}, RC KD27 {ECO:0000313|EMBL:ACM74109.1}, and KR30 RC {ECO:0000313|EMBL:ACM74148.1}; RX PubMed=19624810; DOI=10.1186/1471-2148-9-173; RA Kumar S., Ravuri R.R., Koneru P., Urade B.P., Sarkar B.N., Chandrasekar A., RA Rao V.R.; RT "Reconstructing Indian-Australian phylogenetic link."; RL BMC Evol. Biol. 9:173-173(2009). RN [10] {ECO:0000313|EMBL:ACM74096.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=KD10 {ECO:0000313|EMBL:ACM74122.1}, KD20 RC {ECO:0000313|EMBL:ACM74096.1}, KD23 {ECO:0000313|EMBL:ACM74135.1}, RC KD27 {ECO:0000313|EMBL:ACM74109.1}, and KR30 RC {ECO:0000313|EMBL:ACM74148.1}; 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., Kiran U., RA Gangopadhyay P., Sahani R., Prasad B.V., Gangopadhyay S., Lakshmi G.R., RA Ravuri R.R., Padmaja K., Venugopal P.N., Sharma M.B., 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] {ECO:0000313|EMBL:ACQ75285.1} 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 and D RT in northern Asia."; RL PLoS ONE 5:E15214-E15214(2010). RN [12] {ECO:0000313|EMBL:ADK73027.1} 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 Himalayas."; RL Submitted (MAR-2010) to the EMBL/GenBank/DDBJ databases. RN [13] {ECO:0000313|EMBL:ADM61076.1} 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., 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 [14] {ECO:0000313|EMBL:ADL31534.1} 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 [15] {ECO:0000313|EMBL:AEP23517.1} 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 mtDNA RT lineage."; RL Submitted (JUL-2011) to the EMBL/GenBank/DDBJ databases. RN [16] {ECO:0000313|EMBL:AFF95836.1} 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., Silva N.M., RA Kivisild T., Torroni A., Villems R.; RT "A "Copernican" reassessment of the human mitochondrial DNA tree from its RT root."; RL Am. J. Hum. Genet. 90:675-684(2012). RN [17] {ECO:0000313|EMBL:AFI53413.1} 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., Reidla M., RA Tamm E., Karmin M., Reisberg T., Hooshiar Kashani B., Perego U.A., RA Carossa V., Gandini F., Pereira J.B., Soares P., Angerhofer N., Rychkov S., RA Al-Zahery N., Carelli V., Sanati M.H., Houshmand M., Hatina J., RA Macaulay V., Pereira L., Woodward S.R., Davies W., Gamble C., Baird D., RA Semino O., Villems R., Torroni A., Richards M.B.; RT "Mitochondrial DNA signals of late glacial recolonization of europe from RT near eastern refugia."; RL Am. J. Hum. Genet. 90:915-924(2012). RN [18] {ECO:0000313|EMBL:AET09349.1} 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 rarely RT found in populations of northern Asia and eastern europe."; RL PLoS ONE 7:E32179-E32179(2012). RN [19] {ECO:0000313|EMBL:AFV54003.1} RP NUCLEOTIDE SEQUENCE. RC TISSUE=Saliva {ECO:0000313|EMBL:AFV54003.1}; 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 [20] {ECO:0000313|EMBL:AGX89825.1} RP NUCLEOTIDE SEQUENCE. RA Loo J.-H., Trejaut J.A., Yen J.-C., Chen Z.-S., Ng W.-M., Huang C.-Y., RA Hsu K.-N., Hung K.-H., Hsiao Y., Wei Y.-H., Lin M.; RL Submitted (DEC-2012) to the EMBL/GenBank/DDBJ databases. RN [21] {ECO:0000313|EMBL:AGH18584.1} 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., Carracedo A., RA Torres-Balanza A., Rocabado O., Vullo C., Salas A.; RT "The genetic legacy of the pre-colonial period in contemporary bolivians."; RL PLoS ONE 8:E58980-E58980(2013). RN [22] {ECO:0000313|EMBL:AGW55557.1} RP NUCLEOTIDE SEQUENCE. RA Li S., Schierup M.H.; RT "Variation and association to diabetes in 2000 full mtDNA sequences mined RT from an exome study in a Danish population."; RL Submitted (MAY-2013) to the EMBL/GenBank/DDBJ databases. RN [23] {ECO:0000313|EMBL:AGZ05976.1} RP NUCLEOTIDE SEQUENCE. RA Ko A.M.-S., Chen C.-Y., Fu Q., Delfin F., Li M., Chiu H.-L., Stoneking M., RA Ko Y.-C.; RL Submitted (AUG-2013) to the EMBL/GenBank/DDBJ databases. RN [24] {ECO:0000313|EMBL:AFO26880.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=24341918; DOI=10.1111/acel.12186; 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., Remacle J., RA Robine J.M., Schreiber S., Sikora E., Eline Slagboom P., Spazzafumo L., RA Antonietta Stazi M., Toussaint O., Vaupel J.W., Rose G., Majamaa K., RA Perola M., Johnson T.E., Bolund L., Yang H., 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 13:401-407(2014). RN [25] {ECO:0000313|EMBL:AGZ05976.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=24607387; DOI=10.1016/j.ajhg.2014.02.003; RA Ko A.M., Chen C.Y., Fu Q., Delfin F., Li M., Chiu H.L., Stoneking M., RA Ko Y.C.; RT "Early austronesians: into and out of taiwan."; RL Am. J. Hum. Genet. 94:426-436(2014). RN [26] {ECO:0000313|EMBL:AGX89825.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=24713204; DOI=10.1186/1756-0500-7-223; RA Loo J.H., Trejaut J.A., Yen J.C., Chen Z.S., Ng W.M., Huang C.Y., Hsu K.N., RA Hung K.H., Hsiao Y., Wei Y.H., Lin M.; RT "Mitochondrial DNA association study of type 2 diabetes with or without RT ischemic stroke in Taiwan."; RL BMC Res. Notes 7:223-223(2014). RN [27] {ECO:0000313|EMBL:AHE79526.1} RP NUCLEOTIDE SEQUENCE. RC TISSUE=Peripheral blood {ECO:0000313|EMBL:AHE79526.1}; RX PubMed=24498190; 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 Polycythemia."; RL PLoS ONE 9:E87775-E87775(2014). RN [28] {ECO:0000313|EMBL:AKB98897.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=25832481; DOI=10.1007/s00439-015-1547-4; RA Palanichamy M.G., Mitra B., Zhang C.L., Debnath M., Li G.M., Wang H.W., RA Agrawal S., Chaudhuri T.K., Zhang Y.P.; RT "West Eurasian mtDNA lineages in India: an insight into the spread of the RT Dravidian language and the origins of the caste system."; RL Hum. Genet. 134:637-647(2015). RN [29] {ECO:0000313|EMBL:AJT35983.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=25738654; RA Fernandes V., Triska P., Pereira J.B., Alshamali F., Rito T., Machado A., RA Fajkosova Z., Cavadas B., Cerny V., Soares P., Richards M.B., Pereira L.; RT "Genetic stratigraphy of key demographic events in arabia."; RL PLoS ONE 10:E0118625-E0118625(2015). RN [30] {ECO:0000313|EMBL:ALM03422.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=26509580; RA Hernandez C.L., Soares P., Dugoujon J.M., Novelletto A., Rodriguez J.N., RA Rito T., Oliveira M., Melhaoui M., Baali A., Pereira L., Calderon R.; RT "Early Holocenic and Historic mtDNA African Signatures in the Iberian RT Peninsula: The Andalusian Region as a Paradigm."; RL PLoS ONE 10:E0139784-E0139784(2015). RN [31] {ECO:0000313|EMBL:AJF40067.1} RP NUCLEOTIDE SEQUENCE. RA Greenspan B.; RL Submitted (JAN-2015) to the EMBL/GenBank/DDBJ databases. RN [32] {ECO:0000313|EMBL:APD56977.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=27837350; RA Kutanan W., Kampuansai J., Srikummool M., Kangwanpong D., Ghirotto S., RA Brunelli A., Stoneking M.; RT "Complete mitochondrial genomes of Thai and Lao populations indicate an RT ancient origin of Austroasiatic groups and demic diffusion in the spread of RT Tai-Kadai languages."; RL Hum. Genet. 0:0-0(2016). RN [33] {ECO:0000313|EMBL:AMN85910.1} RP NUCLEOTIDE SEQUENCE. RA Llamas B., Fehren-Schmitz L., Valverde G., Soubrier J., Mallick S., RA Rohland N., Nordenfelt S., Valdiosera C., Richards S.M., Rohrlach A., RA Barreto Romero M.I., Flores Espinoza I., Tomasto Cagigao E., RA Watson Jimenez L., Makowski K., Leboreiro Reyna I.S., Mansilla Lory J., RA Ballivian Torrez J.A., Rivera M.A., Burger R.L., Ceruti M.C., Reinhard J., RA Wells R.S., Politis G., Santoro C.M., Standen V.G., Smith C., Reich D., RA Ho S.Y.W., Cooper A., Haak W.; RT "Ancient mitochondrial DNA provides high-resolution timescale of the RT peopling of the Americas."; RL Submitted (JAN-2016) to the EMBL/GenBank/DDBJ databases. RN [34] {ECO:0000313|EMBL:ATP02664.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=29215085; RA Derenko M., Denisova G., Malyarchuk B., Dambueva I., Bazarov B.; RT "Mitogenomic diversity and differentiation of the Buryats."; RL J. Hum. Genet. 0:0-0(2017). RN [35] {ECO:0000313|EMBL:ASR94067.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=28716916; DOI=10.1073/pnas.1704906114; RA Pierron D., Heiske M., Razafindrazaka H., Rakoto I., Rabetokotany N., RA Ravololomanga B., Rakotozafy L.M., Rakotomalala M.M., Razafiarivony M., RA Rasoarifetra B., Raharijesy M.A., Razafindralambo L., Ramilisonina, RA Fanony F., Lejamble S., Thomas O., Mohamed Abdallah A., Rocher C., RA Arachiche A., Tonaso L., Pereda-Loth V., Schiavinato S., Brucato N., RA Ricaut F.X., Kusuma P., Sudoyo H., Ni S., Boland A., Deleuze J.F., RA Beaujard P., Grange P., Adelaar S., Stoneking M., Rakotoarisoa J.A., RA Radimilahy C., Letellier T.; RT "Genomic landscape of human diversity across Madagascar."; RL Proc. Natl. Acad. Sci. U.S.A. 114:E6498-E6506(2017). RN [36] {ECO:0000313|EMBL:ARK15834.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=28387361; DOI=10.1038/srep46044; RA Sahakyan H., Hooshiar Kashani B., Tamang R., Kushniarevich A., Francis A., RA Costa M.D., Pathak A.K., Khachatryan Z., Sharma I., van Oven M., Parik J., RA Hovhannisyan H., Metspalu E., Pennarun E., Karmin M., Tamm E., Tambets K., RA Bahmanimehr A., Reisberg T., Reidla M., Achilli A., Olivieri A., RA Gandini F., Perego U.A., Al-Zahery N., Houshmand M., Sanati M.H., RA Soares P., Rai E., Sarac J., Saric T., Sharma V., Pereira L., Fernandes V., RA Cerny V., Farjadian S., Singh D.P., Azakli H., Ustek D., Ekomasova N., RA Kutuev I., Litvinov S., Bermisheva M., Khusnutdinova E., Rai N., Singh M., RA Singh V.K., Reddy A.G., Tolk H.-V., Cvjetan S., Lauc L.B., Rudan P., RA Michalodimitrakis E.N., Anagnou N.P., Pappa K.I., Golubenko M.V., RA Orekhov V., Borinskaya S.A., Kaldma K., Schauer M.A., Simionescu M., RA Gusar V., Grechanina E., Govindaraj P., Voevoda M., Damba L., Sharma S., RA Singh L., Semino O., Behar D.M., Yepiskoposyan L., Richards M.B., RA Metspalu M., Kivisild T., Thangaraj K., Endicott P., Chaubey G., RA Torroni A., Villems R.; RT "Origin and spread of human mitochondrial DNA haplogroup U7."; RL Sci. Rep. 7:46044-46044(2017). RN [37] {ECO:0000313|EMBL:QBZ74601.1} RP NUCLEOTIDE SEQUENCE. RA Al-Jasmi F.A., Vijayan R., Souid A.-K., Amiri K.; RT "Mitochondrial DNA Variations among Emiratis."; RL Submitted (JUN-2017) to the EMBL/GenBank/DDBJ databases. RN [38] {ECO:0000313|EMBL:ATL17948.1} RP NUCLEOTIDE SEQUENCE. RA Peng M.-S., Song J.-J., Zhang Y.-P.; RT "Mitochondrial Genomes Uncover the Maternal History of the Pamir RT Populations."; RL Submitted (JUL-2017) to the EMBL/GenBank/DDBJ databases. RN [39] {ECO:0000313|EMBL:AZJ13684.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=30594063; DOI=10.1016/j.fsigen.2018.12.007; RA Simao F., Strobl C., Vullo C., Catelli L., Machado P., Huber N., RA Schnaller L., Huber G., Xavier C., Carvalho E.F., Gusmao L., Parson W.; RT "The maternal inheritance of Alto Parana revealed by full mitogenome RT sequences."; RL Forensic Sci. Int. Genet. 39:66-72(2018). RN [40] {ECO:0000313|EMBL:QAT98036.1} RP NUCLEOTIDE SEQUENCE. RC TISSUE=Muscle {ECO:0000313|EMBL:QAT98036.1}; RX PubMed=30528267; RA Saldana-Martinez A., Munoz M.L., Perez-Ramirez G., Montiel-Sosa J.F., RA Montoya J., Emperador S., Ruiz-Pesini E., Cuevas-Covarrubias S., RA Lopez-Valdez J., Ramirez R.G.; RT "Whole sequence of the mitochondrial DNA genome of Kearns Sayre Syndrome RT patients: Identification of deletions and variants."; RL Gene 688:171-181(2018). RN [41] {ECO:0000313|EMBL:AUR53850.1} RP NUCLEOTIDE SEQUENCE. RA Gaut B.S., Morton B.R., Clegg M.T., Duvall M.R.; RL Submitted (JAN-2018) to the EMBL/GenBank/DDBJ databases. RN [42] {ECO:0000313|EMBL:AZJ10287.1} RP NUCLEOTIDE SEQUENCE. RA Piotrowska-Nowak A., Elson J.L., Sobczyk-Kopciol A., Drygas W., RA Piwonska A., Puch-Walczak A., Ploski R., Bartnik E., Tonska K.; RT "New mtDNA association model, MutPred variant load, suggests individuals RT with multiple mildly deleterious mtDNA variants are more likely to suffer RT from atherosclerosis."; RL Submitted (MAR-2018) to the EMBL/GenBank/DDBJ databases. RN [43] {ECO:0000313|EMBL:QXE40396.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=AmiA39 {ECO:0000313|EMBL:QXE40396.1}; RA Reidla M., Metspalu E., Herrera R.; RT "The Ami and Yami aborigines of Taiwan and their genetic relationship to RT East Asian and Pacific populations."; RL Submitted (SEP-2019) to the EMBL/GenBank/DDBJ databases. RN [44] {ECO:0000313|EMBL:QXO91513.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=DH048 {ECO:0000313|EMBL:QXO91513.1}; RA USDA Swine Surveillance A., Lin K., Dunn D., Zhang J., Gauger P.; RL Submitted (OCT-2019) to the EMBL/GenBank/DDBJ databases. RN [45] {ECO:0000313|EMBL:QGW54977.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=32504149; RA Davidovic S., Malyarchuk B., Grzybowski T., Aleksic J.M., Derenko M., RA Litvinov A., Rogalla-Ladniak U., Stevanovic M., Kovacevic-Grujicic N.; RT "Complete mitogenome data for the Serbian population: the contribution to RT high-quality forensic databases."; RL Int. J. Legal Med. 0:0-0(2020). RN [46] {ECO:0000313|EMBL:QKT25988.1} RP NUCLEOTIDE SEQUENCE. RX PubMed=32483274; RA Pedro N., Brucato N., Fernandes V., Andre M., Saag L., Pomat W., Besse C., RA Boland A., Deleuze J.F., Clarkson C., Sudoyo H., Metspalu M., Stoneking M., RA Cox M.P., Leavesley M., Pereira L., Ricaut F.X.; RT "Papuan mitochondrial genomes and the settlement of Sahul."; RL J. Hum. Genet. 0:0-0(2020). RN [47] {ECO:0000313|EMBL:QVD40193.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=ASO_B12_S19 {ECO:0000313|EMBL:QVD40193.1}; RA Roca-Rada X., Politis G., Messineo P., Scheifler N., Scabuzzo C., RA Gonzalez M., Harkins K.M., Reich D., Souilmi Y., Teixeira J.C., Llamas B., RA Fehren-Schmitz L.; RT "New insights on the peopling of the Southern Cone of South America from RT Early to Late Holocene mitochondrial genomes from the Argentinian Pampas."; RL Submitted (NOV-2020) to the EMBL/GenBank/DDBJ databases. RN [48] {ECO:0000313|EMBL:QRC37185.1} RP NUCLEOTIDE SEQUENCE. RC STRAIN=Pol-5 {ECO:0000313|EMBL:QRC37185.1}; RC TISSUE=Bone tissue {ECO:0000313|EMBL:QRC37185.1}; RA Diroma M.A., Modi A., Lari M., Sineo L., Caramelli D., Vai S.; RT "New insights into mitochondrial DNA reconstruction and variant detection RT in ancient samples."; RL Submitted (DEC-2020) to the EMBL/GenBank/DDBJ databases. CC -!- FUNCTION: Component of the cytochrome c oxidase, the last enzyme in the CC mitochondrial electron transport chain which drives oxidative CC phosphorylation. The respiratory chain contains 3 multisubunit CC complexes succinate dehydrogenase (complex II, CII), ubiquinol- CC cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CC CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to CC transfer electrons derived from NADH and succinate to molecular oxygen, CC creating an electrochemical gradient over the inner membrane that CC drives transmembrane transport and the ATP synthase. Cytochrome c CC oxidase is the component of the respiratory chain that catalyzes the CC reduction of oxygen to water. Electrons originating from reduced CC cytochrome c in the intermembrane space (IMS) are transferred via the CC dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 CC to the active site in subunit 1, a binuclear center (BNC) formed by CC heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 CC water molecules using 4 electrons from cytochrome c in the IMS and 4 CC protons from the mitochondrial matrix. {ECO:0000256|RuleBase:RU000369}. CC -!- CATALYTIC ACTIVITY: CC Reaction=4 Fe(II)-[cytochrome c] + 8 H(+)(in) + O2 = 4 Fe(III)- CC [cytochrome c] + 4 H(+)(out) + 2 H2O; Xref=Rhea:RHEA:11436, CC Rhea:RHEA-COMP:10350, Rhea:RHEA-COMP:14399, ChEBI:CHEBI:15377, CC ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:29033, CC ChEBI:CHEBI:29034; EC=7.1.1.9; CC Evidence={ECO:0000256|ARBA:ARBA00029331}; CC PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:11437; CC Evidence={ECO:0000256|ARBA:ARBA00029331}; CC -!- COFACTOR: CC Name=Cu cation; Xref=ChEBI:CHEBI:23378; CC Evidence={ECO:0000256|ARBA:ARBA00001935}; CC -!- COFACTOR: CC Name=heme; Xref=ChEBI:CHEBI:30413; CC Evidence={ECO:0000256|ARBA:ARBA00001971}; CC -!- PATHWAY: Energy metabolism; oxidative phosphorylation. CC {ECO:0000256|ARBA:ARBA00004673, ECO:0000256|RuleBase:RU000369}. CC -!- SUBCELLULAR LOCATION: Membrane {ECO:0000256|ARBA:ARBA00004141}; Multi- CC pass membrane protein {ECO:0000256|ARBA:ARBA00004141}. Mitochondrion CC inner membrane {ECO:0000256|RuleBase:RU000369}; Multi-pass membrane CC protein {ECO:0000256|RuleBase:RU000369}. CC -!- SIMILARITY: Belongs to the heme-copper respiratory oxidase family. CC {ECO:0000256|ARBA:ARBA00009578, ECO:0000256|RuleBase:RU000369}. 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; KF161153; AGW55557.1; -; Genomic_DNA. DR EMBL; KF162590; AGW74237.1; -; Genomic_DNA. DR EMBL; KC252342; AGX89825.1; -; Genomic_DNA. DR EMBL; KC252360; AGX90059.1; -; Genomic_DNA. DR EMBL; KC252414; AGX90761.1; -; Genomic_DNA. DR EMBL; KC252422; AGX90865.1; -; Genomic_DNA. DR EMBL; KC252441; AGX91112.1; -; Genomic_DNA. DR EMBL; KC252445; AGX91164.1; -; Genomic_DNA. DR EMBL; KC252471; AGX91502.1; -; Genomic_DNA. DR EMBL; KC252495; AGX91814.1; -; Genomic_DNA. DR EMBL; KC252540; AGX92399.1; -; Genomic_DNA. DR EMBL; KF540514; AGZ05976.1; -; Genomic_DNA. DR EMBL; KF540524; AGZ06106.1; -; Genomic_DNA. DR EMBL; KF540532; AGZ06210.1; -; Genomic_DNA. DR EMBL; KF540543; AGZ06353.1; -; Genomic_DNA. DR EMBL; KF540549; AGZ06431.1; -; Genomic_DNA. DR EMBL; KF540809; AGZ09811.1; -; Genomic_DNA. DR EMBL; KF849972; AHE79526.1; -; Genomic_DNA. DR EMBL; KP691986; AJF40067.1; -; Genomic_DNA. DR EMBL; KP317029; AJT35983.1; -; Genomic_DNA. DR EMBL; KP763847; AKB98897.1; -; Genomic_DNA. DR EMBL; KT819221; ALM03422.1; -; Genomic_DNA. DR EMBL; KU523269; AMN85910.1; -; Genomic_DNA. DR EMBL; KX456765; APD56977.1; -; Genomic_DNA. DR EMBL; KX456886; APD58549.1; -; Genomic_DNA. DR EMBL; KX457378; APD64945.1; -; Genomic_DNA. DR EMBL; KX457392; APD65127.1; -; Genomic_DNA. DR EMBL; KY824881; ARK15834.1; -; Genomic_DNA. DR EMBL; MF055780; ASR94067.1; -; Genomic_DNA. DR EMBL; MF055957; ASR96368.1; -; Genomic_DNA. DR EMBL; MF056587; ASS04560.1; -; Genomic_DNA. DR EMBL; MF056669; ASS05626.1; -; Genomic_DNA. DR EMBL; MF056768; ASS06900.1; -; Genomic_DNA. DR EMBL; MF056810; ASS07446.1; -; Genomic_DNA. DR EMBL; MF056818; ASS07550.1; -; Genomic_DNA. DR EMBL; MF056843; ASS07875.1; -; Genomic_DNA. DR EMBL; MF057119; ASS11451.1; -; Genomic_DNA. DR EMBL; MF057332; ASS14220.1; -; Genomic_DNA. DR EMBL; MF057848; ASS21111.1; -; Genomic_DNA. DR EMBL; MF057856; ASS21215.1; -; Genomic_DNA. DR EMBL; MF057862; ASS21293.1; -; Genomic_DNA. DR EMBL; MF057994; ASS23009.1; -; Genomic_DNA. DR EMBL; MF058081; ASS24140.1; -; Genomic_DNA. DR EMBL; MF058375; ASS27949.1; -; Genomic_DNA. DR EMBL; MF522911; ATL17948.1; -; Genomic_DNA. DR EMBL; MF522936; ATL18273.1; -; Genomic_DNA. DR EMBL; MF043513; ATP02664.1; -; Genomic_DNA. DR EMBL; MG767170; AUR53850.1; -; Genomic_DNA. DR EMBL; MH120591; AZJ10287.1; -; Genomic_DNA. DR EMBL; MH981824; AZJ13684.1; -; Genomic_DNA. DR EMBL; MG652751; QAT98036.1; -; Genomic_DNA. DR EMBL; MF437105; QBZ74601.1; -; Genomic_DNA. DR EMBL; MF437160; QBZ75316.1; -; Genomic_DNA. DR EMBL; MF437194; QBZ75758.1; -; Genomic_DNA. DR EMBL; MF437258; QBZ76590.1; -; Genomic_DNA. DR EMBL; MF437267; QBZ76707.1; -; Genomic_DNA. DR EMBL; MK134360; QGW54977.1; -; Genomic_DNA. DR EMBL; MN849804; QKT25988.1; -; Genomic_DNA. DR EMBL; MW389270; QRC37185.1; -; Genomic_DNA. DR EMBL; MW291666; QVD40193.1; -; Genomic_DNA. DR EMBL; MN482524; QXE40396.1; -; Genomic_DNA. DR EMBL; MN628073; QXO91513.1; -; Genomic_DNA. DR AlphaFoldDB; Q6VHV2; -. DR SMR; Q6VHV2; -. DR PeptideAtlas; Q6VHV2; -. DR UniPathway; UPA00705; -. DR GO; GO:0005743; C:mitochondrial inner membrane; IEA:UniProtKB-SubCell. DR GO; GO:0045277; C:respiratory chain complex IV; IEA:InterPro. DR GO; GO:0004129; F:cytochrome-c oxidase activity; IEA:UniProtKB-EC. DR GO; GO:0020037; F:heme binding; IEA:InterPro. DR GO; GO:0046872; F:metal ion binding; IEA:UniProtKB-KW. DR GO; GO:0006119; P:oxidative phosphorylation; IEA:UniProtKB-UniPathway. DR CDD; cd01663; Cyt_c_Oxidase_I; 1. DR Gene3D; 1.20.210.10; Cytochrome c oxidase-like, subunit I domain; 1. DR InterPro; IPR023616; Cyt_c_oxase-like_su1_dom. DR InterPro; IPR036927; Cyt_c_oxase-like_su1_sf. DR InterPro; IPR000883; Cyt_C_Oxase_1. DR InterPro; IPR023615; Cyt_c_Oxase_su1_BS. DR InterPro; IPR033944; Cyt_c_oxase_su1_dom. DR PANTHER; PTHR10422; CYTOCHROME C OXIDASE SUBUNIT 1; 1. DR PANTHER; PTHR10422:SF18; CYTOCHROME C OXIDASE SUBUNIT 1; 1. DR Pfam; PF00115; COX1; 1. DR PRINTS; PR01165; CYCOXIDASEI. DR SUPFAM; SSF81442; Cytochrome c oxidase subunit I-like; 1. DR PROSITE; PS50855; COX1; 1. DR PROSITE; PS00077; COX1_CUB; 1. PE 3: Inferred from homology; KW Calcium {ECO:0000256|ARBA:ARBA00022837}; KW Copper {ECO:0000256|ARBA:ARBA00023008, ECO:0000256|RuleBase:RU000369}; KW Electron transport {ECO:0000256|RuleBase:RU000369}; KW Heme {ECO:0000256|RuleBase:RU000369}; Iron {ECO:0000256|RuleBase:RU000369}; KW Membrane {ECO:0000256|ARBA:ARBA00023136, ECO:0000256|RuleBase:RU000369}; KW Metal-binding {ECO:0000256|RuleBase:RU000369}; KW Mitochondrion {ECO:0000256|RuleBase:RU000369, ECO:0000313|EMBL:AAP91157.1}; KW Mitochondrion inner membrane {ECO:0000256|ARBA:ARBA00022792, KW ECO:0000256|RuleBase:RU000369}; KW Respiratory chain {ECO:0000256|RuleBase:RU000369}; KW Sodium {ECO:0000256|ARBA:ARBA00023053}; KW Transmembrane {ECO:0000256|ARBA:ARBA00022692, KW ECO:0000256|RuleBase:RU000369}; KW Transmembrane helix {ECO:0000256|ARBA:ARBA00022989, KW ECO:0000256|SAM:Phobius}; Transport {ECO:0000256|RuleBase:RU000369}. FT TRANSMEM 18..37 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 57..83 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 104..126 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 146..171 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 183..210 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 243..261 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 268..291 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 303..325 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 337..359 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 379..400 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 412..430 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT TRANSMEM 450..473 FT /note="Helical" FT /evidence="ECO:0000256|SAM:Phobius" FT DOMAIN 1..511 FT /note="Cytochrome oxidase subunit I profile" FT /evidence="ECO:0000259|PROSITE:PS50855" 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 //