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Protein

Cytochrome c oxidase subunit 1

Gene

MT-CO1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B.

Catalytic activityi

4 ferrocytochrome c + O2 + 4 H+ = 4 ferricytochrome c + 2 H2O.

Pathwayi: oxidative phosphorylation

This protein is involved in the pathway oxidative phosphorylation, which is part of Energy metabolism.
View all proteins of this organism that are known to be involved in the pathway oxidative phosphorylation and in Energy metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi61Iron (heme A axial ligand)Curated1
Metal bindingi240Copper BCurated1
Metal bindingi244Copper BCurated1
Metal bindingi290Copper BCurated1
Metal bindingi291Copper BCurated1
Metal bindingi376Iron (heme A3 axial ligand)Curated1
Metal bindingi378Iron (heme A axial ligand)Curated1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
Biological processElectron transport, Respiratory chain, Transport
LigandCopper, Heme, Iron, Metal-binding

Enzyme and pathway databases

BRENDAi1.9.3.1 2681
ReactomeiR-HSA-5628897 TP53 Regulates Metabolic Genes
R-HSA-611105 Respiratory electron transport
UniPathwayiUPA00705

Protein family/group databases

TCDBi3.D.4.11.1 the proton-translocating cytochrome oxidase (cox) superfamily

Names & Taxonomyi

Protein namesi
Recommended name:
Cytochrome c oxidase subunit 1 (EC:1.9.3.1)
Alternative name(s):
Cytochrome c oxidase polypeptide I
Gene namesi
Name:MT-CO1
Synonyms:COI, COXI, MTCO1
Encoded oniMitochondrion
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Mitochondrion

Organism-specific databases

EuPathDBiHostDB:ENSG00000198804.2
HGNCiHGNC:7419 MT-CO1
MIMi516030 gene
neXtProtiNX_P00395

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 19Mitochondrial matrixSequence analysisAdd BLAST19
Transmembranei20 – 42Helical; Name=1Sequence analysisAdd BLAST23
Topological domaini43 – 56Mitochondrial intermembraneSequence analysisAdd BLAST14
Transmembranei57 – 79Helical; Name=2Sequence analysisAdd BLAST23
Topological domaini80 – 99Mitochondrial matrixSequence analysisAdd BLAST20
Transmembranei100 – 122Helical; Name=3Sequence analysisAdd BLAST23
Topological domaini123 – 146Mitochondrial intermembraneSequence analysisAdd BLAST24
Transmembranei147 – 169Helical; Name=4Sequence analysisAdd BLAST23
Topological domaini170 – 181Mitochondrial matrixSequence analysisAdd BLAST12
Transmembranei182 – 204Helical; Name=5Sequence analysisAdd BLAST23
Topological domaini205 – 235Mitochondrial intermembraneSequence analysisAdd BLAST31
Transmembranei236 – 258Helical; Name=6Sequence analysisAdd BLAST23
Topological domaini259 – 270Mitochondrial matrixSequence analysisAdd BLAST12
Transmembranei271 – 293Helical; Name=7Sequence analysisAdd BLAST23
Topological domaini294 – 302Mitochondrial intermembraneSequence analysis9
Transmembranei303 – 325Helical; Name=8Sequence analysisAdd BLAST23
Topological domaini326 – 336Mitochondrial matrixSequence analysisAdd BLAST11
Transmembranei337 – 359Helical; Name=9Sequence analysisAdd BLAST23
Topological domaini360 – 373Mitochondrial intermembraneSequence analysisAdd BLAST14
Transmembranei374 – 396Helical; Name=10Sequence analysisAdd BLAST23
Topological domaini397 – 408Mitochondrial matrixSequence analysisAdd BLAST12
Transmembranei409 – 431Helical; Name=11Sequence analysisAdd BLAST23
Topological domaini432 – 450Mitochondrial intermembraneSequence analysisAdd BLAST19
Transmembranei451 – 473Helical; Name=12Sequence analysisAdd BLAST23
Topological domaini474 – 513Mitochondrial matrixSequence analysisAdd BLAST40

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Leber hereditary optic neuropathy (LHON)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
See also OMIM:535000
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_008387513S → SKQK in LHON; secondary mutation; does not seem to directly cause the disease. 1 Publication1
MT-CO1 may play a role in the pathogenesis of acquired idiopathic sideroblastic anemia, a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria. Mitochondrial iron overload may be attributable to mutations of mitochondrial DNA because these can cause respiratory chain dysfunction, thereby impairing reduction of ferric iron to ferrous iron. The reduced form of iron is essential to the last step of mitochondrial heme biosynthesis.2 Publications
Mitochondrial complex IV deficiency (MT-C4D)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, exercise intolerance, developmental delay, delayed motor development and mental retardation. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome.
See also OMIM:220110
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_033055142S → F in MT-C4D; significant decrease in enzyme activity. 1 PublicationCorresponds to variant dbSNP:rs267606883Ensembl.1
Natural variantiVAR_033056196L → I in MT-C4D. 1 PublicationCorresponds to variant dbSNP:rs28461189Ensembl.1
Recurrent myoglobinuria mitochondrial (RM-MT)1 Publication
The gene represented in this entry may be involved in disease pathogenesis.
Disease descriptionRecurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine.
See also OMIM:550500
Deafness, sensorineural, mitochondrial (DFNM)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of non-syndromic deafness with maternal inheritance. Affected individuals manifest progressive, postlingual, sensorineural hearing loss involving high frequencies.
See also OMIM:500008
Colorectal cancer (CRC)2 Publications
The gene represented in this entry may be involved in disease pathogenesis.
Disease descriptionA complex disease characterized by malignant lesions arising from the inner wall of the large intestine (the colon) and the rectum. Genetic alterations are often associated with progression from premalignant lesion (adenoma) to invasive adenocarcinoma. Risk factors for cancer of the colon and rectum include colon polyps, long-standing ulcerative colitis, and genetic family history.
See also OMIM:114500
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_064154125G → D in CRC; displays steady-state catalytic activity linked to proton pumping that is approximately 34% of wild-type; an intrinsic proton leak is find in the enzyme, which will lead to decreased overall energy-conversion efficiency of the respiratory chain, perturbing transport processes such as protein, ion and metabolite trafficking. 2 PublicationsCorresponds to variant dbSNP:rs281865417Ensembl.1
Natural variantiVAR_064155458S → P in CRC; the mutant is probably not expressed, indicating that the amino acid substitution results in a severely altered overall structure of the enzyme. 2 PublicationsCorresponds to variant dbSNP:rs267606884Ensembl.1

Keywords - Diseasei

Deafness, Disease mutation, Leber hereditary optic neuropathy, Non-syndromic deafness, Primary mitochondrial disease

Organism-specific databases

DisGeNETi4512
GeneReviewsiMT-CO1
MalaCardsiMT-CO1
MIMi114500 phenotype
220110 phenotype
500008 phenotype
535000 phenotype
550500 phenotype
OpenTargetsiENSG00000198804
Orphaneti99845 Genetic recurrent myoglobinuria
254905 Isolated cytochrome C oxidase deficiency
104 Leber hereditary optic neuropathy
255210 Maternally-inherited Leigh syndrome
550 MELAS
90641 Mitochondrial non-syndromic sensorineural deafness
168609 Mitochondrial non-syndromic sensorineural deafness with susceptibility to aminoglycoside exposure
PharmGKBiPA31225

Chemistry databases

ChEMBLiCHEMBL6173
DrugBankiDB02659 Cholic Acid
DB04464 N-Formylmethionine
DB09325 Sodium fluoride

Polymorphism and mutation databases

DMDMi116977

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001833451 – 513Cytochrome c oxidase subunit 1Add BLAST513

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki240 ↔ 2441'-histidyl-3'-tyrosine (His-Tyr)By similarity

Proteomic databases

EPDiP00395
PaxDbiP00395
PeptideAtlasiP00395
PRIDEiP00395
TopDownProteomicsiP00395

PTM databases

iPTMnetiP00395
PhosphoSitePlusiP00395

Expressioni

Gene expression databases

BgeeiENSG00000198804
ExpressionAtlasiP00395 baseline and differential
GenevisibleiP00395 HS

Organism-specific databases

HPAiCAB016241
HPA069328

Interactioni

Subunit structurei

As a newly synthesized protein, rapidly incorporates into a multi-subunit assembly intermediate in the inner membrane, called MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase) complex, whose core components are COA3/MITRAC12 and COX14. Within the MITRAC complex, interacts with COA3 and with SMIM20/MITRAC7; the interaction with SMIM20 stabilizes the newly synthesized MT-CO1 and prevents its premature turnover (PubMed:26321642). Interacts with TMEM177 in a COX20-dependent manner (PubMed:29154948).2 Publications

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi110615, 36 interactors
CORUMiP00395
IntActiP00395, 15 interactors
MINTiP00395
STRINGi9606.ENSP00000354499

Chemistry databases

BindingDBiP00395

Structurei

3D structure databases

ProteinModelPortaliP00395
SMRiP00395
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG4769 Eukaryota
COG0843 LUCA
GeneTreeiENSGT00390000001518
HOVERGENiHBG003841
InParanoidiP00395
KOiK02256
OMAiMSFWLLP
OrthoDBiEOG091G035B
PhylomeDBiP00395
TreeFamiTF353096

Family and domain databases

CDDicd01663 Cyt_c_Oxidase_I, 1 hit
Gene3Di1.20.210.10, 1 hit
InterProiView protein in InterPro
IPR023616 Cyt_c_oxase-like_su1_dom
IPR036927 Cyt_c_oxase-like_su1_sf
IPR000883 Cyt_C_Oxase_1
IPR023615 Cyt_c_Oxase_su1_BS
IPR033944 Cyt_c_oxase_su1_dom
PANTHERiPTHR10422 PTHR10422, 1 hit
PfamiView protein in Pfam
PF00115 COX1, 1 hit
PRINTSiPR01165 CYCOXIDASEI
SUPFAMiSSF81442 SSF81442, 1 hit
PROSITEiView protein in PROSITE
PS50855 COX1, 1 hit
PS00077 COX1_CUB, 1 hit

Sequencei

Sequence statusi: Complete.

P00395-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MFADRWLFST NHKDIGTLYL LFGAWAGVLG TALSLLIRAE LGQPGNLLGN
60 70 80 90 100
DHIYNVIVTA HAFVMIFFMV MPIMIGGFGN WLVPLMIGAP DMAFPRMNNM
110 120 130 140 150
SFWLLPPSLL LLLASAMVEA GAGTGWTVYP PLAGNYSHPG ASVDLTIFSL
160 170 180 190 200
HLAGVSSILG AINFITTIIN MKPPAMTQYQ TPLFVWSVLI TAVLLLLSLP
210 220 230 240 250
VLAAGITMLL TDRNLNTTFF DPAGGGDPIL YQHLFWFFGH PEVYILILPG
260 270 280 290 300
FGMISHIVTY YSGKKEPFGY MGMVWAMMSI GFLGFIVWAH HMFTVGMDVD
310 320 330 340 350
TRAYFTSATM IIAIPTGVKV FSWLATLHGS NMKWSAAVLW ALGFIFLFTV
360 370 380 390 400
GGLTGIVLAN SSLDIVLHDT YYVVAHFHYV LSMGAVFAIM GGFIHWFPLF
410 420 430 440 450
SGYTLDQTYA KIHFTIMFIG VNLTFFPQHF LGLSGMPRRY SDYPDAYTTW
460 470 480 490 500
NILSSVGSFI SLTAVMLMIF MIWEAFASKR KVLMVEEPSM NLEWLYGCPP
510
PYHTFEEPVY MKS
Length:513
Mass (Da):57,041
Last modified:July 21, 1986 - v1
Checksum:iDBCBFE808650AE0D
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_00856610T → A1 Publication1
Natural variantiVAR_00856794F → C1 Publication1
Natural variantiVAR_064154125G → D in CRC; displays steady-state catalytic activity linked to proton pumping that is approximately 34% of wild-type; an intrinsic proton leak is find in the enzyme, which will lead to decreased overall energy-conversion efficiency of the respiratory chain, perturbing transport processes such as protein, ion and metabolite trafficking. 2 PublicationsCorresponds to variant dbSNP:rs281865417Ensembl.1
Natural variantiVAR_033055142S → F in MT-C4D; significant decrease in enzyme activity. 1 PublicationCorresponds to variant dbSNP:rs267606883Ensembl.1
Natural variantiVAR_008568155V → L1 Publication1
Natural variantiVAR_033056196L → I in MT-C4D. 1 PublicationCorresponds to variant dbSNP:rs28461189Ensembl.1
Natural variantiVAR_008569224G → A1 Publication1
Natural variantiVAR_011342235F → S1 PublicationCorresponds to variant dbSNP:rs2853818Ensembl.1
Natural variantiVAR_008385273M → T Found in two patients with acquired idiopathic sideroblastic anemia. 2 PublicationsCorresponds to variant dbSNP:rs199476127Ensembl.1
Natural variantiVAR_008386280I → T Found in two patients with acquired idiopathic sideroblastic anemia. 2 PublicationsCorresponds to variant dbSNP:rs199476126Ensembl.1
Natural variantiVAR_008570305F → L1 PublicationCorresponds to variant dbSNP:rs368552121Ensembl.1
Natural variantiVAR_011343415T → A1 PublicationCorresponds to variant dbSNP:rs372136420Ensembl.1
Natural variantiVAR_064155458S → P in CRC; the mutant is probably not expressed, indicating that the amino acid substitution results in a severely altered overall structure of the enzyme. 2 PublicationsCorresponds to variant dbSNP:rs267606884Ensembl.1
Natural variantiVAR_008387513S → SKQK in LHON; secondary mutation; does not seem to directly cause the disease. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
V00662 Genomic DNA Translation: CAA24028.1
J01415 Genomic DNA Translation: AAB58945.1
D38112 Genomic DNA Translation: BAA07292.1
AY339402 Genomic DNA Translation: AAP89038.1
AY339403 Genomic DNA Translation: AAP89051.1
AY339404 Genomic DNA Translation: AAP89064.1
AY339405 Genomic DNA Translation: AAP89077.1
AY339406 Genomic DNA Translation: AAP89090.1
AY339407 Genomic DNA Translation: AAP89103.1
AY339408 Genomic DNA Translation: AAP89116.1
AY339409 Genomic DNA Translation: AAP89129.1
AY339410 Genomic DNA Translation: AAP89142.1
AY339411 Genomic DNA Translation: AAP89155.1
AY339412 Genomic DNA Translation: AAP89168.1
AY339421 Genomic DNA Translation: AAP89285.1
AY339422 Genomic DNA Translation: AAP89298.1
AY339423 Genomic DNA Translation: AAP89311.1
AY339424 Genomic DNA Translation: AAP89324.1
AY339425 Genomic DNA Translation: AAP89337.1
AY339426 Genomic DNA Translation: AAP89350.1
AY339427 Genomic DNA Translation: AAP89363.1
AY339428 Genomic DNA Translation: AAP89376.1
AY339429 Genomic DNA Translation: AAP89389.1
AY339430 Genomic DNA Translation: AAP89402.1
AY339431 Genomic DNA Translation: AAP89415.1
AY339432 Genomic DNA Translation: AAP89428.1
AY339433 Genomic DNA Translation: AAP89441.1
AY339434 Genomic DNA Translation: AAP89454.1
AY339435 Genomic DNA Translation: AAP89467.1
AY339436 Genomic DNA Translation: AAP89480.1
AY339437 Genomic DNA Translation: AAP89493.1
AY339438 Genomic DNA Translation: AAP89506.1
AY339439 Genomic DNA Translation: AAP89519.1
AY339440 Genomic DNA Translation: AAP89532.1
AY339441 Genomic DNA Translation: AAP89545.1
AY339442 Genomic DNA Translation: AAP89558.1
AY339443 Genomic DNA Translation: AAP89571.1
AY339444 Genomic DNA Translation: AAP89584.1
AY339445 Genomic DNA Translation: AAP89597.1
AY339451 Genomic DNA Translation: AAP89675.1
AY339452 Genomic DNA Translation: AAP89688.1
AY339453 Genomic DNA Translation: AAP89701.1
AY339454 Genomic DNA Translation: AAP89714.1
AY339455 Genomic DNA Translation: AAP89727.1
AY339456 Genomic DNA Translation: AAP89740.1
AY339457 Genomic DNA Translation: AAP89753.1
AY339458 Genomic DNA Translation: AAP89766.1
AY339459 Genomic DNA Translation: AAP89779.1
AY339460 Genomic DNA Translation: AAP89792.1
AY339461 Genomic DNA Translation: AAP89805.1
AY339462 Genomic DNA Translation: AAP89818.1
AY339463 Genomic DNA Translation: AAP89831.1
AY339464 Genomic DNA Translation: AAP89844.1
AY339465 Genomic DNA Translation: AAP89857.1
AY339466 Genomic DNA Translation: AAP89870.1
AY339467 Genomic DNA Translation: AAP89883.1
AY339468 Genomic DNA Translation: AAP89896.1
AY339469 Genomic DNA Translation: AAP89909.1
AY339470 Genomic DNA Translation: AAP89922.1
AY339471 Genomic DNA Translation: AAP89935.1
AY339472 Genomic DNA Translation: AAP89948.1
AY339473 Genomic DNA Translation: AAP89961.1
AY339474 Genomic DNA Translation: AAP89974.1
AY339475 Genomic DNA Translation: AAP89987.1
AY339476 Genomic DNA Translation: AAP90000.1
AY339477 Genomic DNA Translation: AAP90013.1
AY339478 Genomic DNA Translation: AAP90026.1
AY339479 Genomic DNA Translation: AAP90039.1
AY339480 Genomic DNA Translation: AAP90052.1
AY339481 Genomic DNA Translation: AAP90065.1
AY339482 Genomic DNA Translation: AAP90078.1
AY339483 Genomic DNA Translation: AAP90091.1
AY339484 Genomic DNA Translation: AAP90104.1
AY339485 Genomic DNA Translation: AAP90117.1
AY339486 Genomic DNA Translation: AAP90130.1
AY339487 Genomic DNA Translation: AAP90143.1
AY339488 Genomic DNA Translation: AAP90156.1
AY339489 Genomic DNA Translation: AAP90169.1
AY339490 Genomic DNA Translation: AAP90182.1
AY339491 Genomic DNA Translation: AAP90195.1
AY339492 Genomic DNA Translation: AAP90208.1
AY339493 Genomic DNA Translation: AAP90221.1
AY339494 Genomic DNA Translation: AAP90234.1
AY339495 Genomic DNA Translation: AAP90247.1
AY339496 Genomic DNA Translation: AAP90260.1
AY339497 Genomic DNA Translation: AAP90273.1
AY339498 Genomic DNA Translation: AAP90286.1
AY339499 Genomic DNA Translation: AAP90299.1
AY339500 Genomic DNA Translation: AAP90312.1
AY339501 Genomic DNA Translation: AAP90325.1
AY339502 Genomic DNA Translation: AAP90338.1
AY339503 Genomic DNA Translation: AAP90351.1
AY339504 Genomic DNA Translation: AAP90364.1
AY339505 Genomic DNA Translation: AAP90377.1
AY339506 Genomic DNA Translation: AAP90390.1
AY339507 Genomic DNA Translation: AAP90403.1
AY339508 Genomic DNA Translation: AAP90416.1
AY339509 Genomic DNA Translation: AAP90429.1
AY339510 Genomic DNA Translation: AAP90442.1
AY339511 Genomic DNA Translation: AAP90455.1
AY339512 Genomic DNA Translation: AAP90468.1
AY339513 Genomic DNA Translation: AAP90481.1
AY339514 Genomic DNA Translation: AAP90494.1
AY339515 Genomic DNA Translation: AAP90507.1
AY339516 Genomic DNA Translation: AAP90520.1
AY339517 Genomic DNA Translation: AAP90533.1
AY339518 Genomic DNA Translation: AAP90546.1
AY339519 Genomic DNA Translation: AAP90559.1
AY339520 Genomic DNA Translation: AAP90572.1
AY339521 Genomic DNA Translation: AAP90585.1
AY339522 Genomic DNA Translation: AAP90598.1
AY339523 Genomic DNA Translation: AAP90611.1
AY339524 Genomic DNA Translation: AAP90624.1
AY339525 Genomic DNA Translation: AAP90637.1
AY339526 Genomic DNA Translation: AAP90650.1
AY339527 Genomic DNA Translation: AAP90663.1
AY339528 Genomic DNA Translation: AAP90676.1
AY339529 Genomic DNA Translation: AAP90689.1
AY339530 Genomic DNA Translation: AAP90702.1
AY339531 Genomic DNA Translation: AAP90715.1
AY339532 Genomic DNA Translation: AAP90728.1
AY339533 Genomic DNA Translation: AAP90741.1
AY339534 Genomic DNA Translation: AAP90754.1
AY339535 Genomic DNA Translation: AAP90767.1
AY339536 Genomic DNA Translation: AAP90780.1
AY339537 Genomic DNA Translation: AAP90793.1
AY339538 Genomic DNA Translation: AAP90806.1
AY339539 Genomic DNA Translation: AAP90819.1
AY339540 Genomic DNA Translation: AAP90832.1
AY339541 Genomic DNA Translation: AAP90845.1
AY339542 Genomic DNA Translation: AAP90858.1
AY339543 Genomic DNA Translation: AAP90871.1
AY339544 Genomic DNA Translation: AAP90884.1
AY339545 Genomic DNA Translation: AAP90897.1
AY339546 Genomic DNA Translation: AAP90910.1
AY339547 Genomic DNA Translation: AAP90923.1
AY339548 Genomic DNA Translation: AAP90936.1
AY339549 Genomic DNA Translation: AAP90949.1
AY339550 Genomic DNA Translation: AAP90962.1
AY339551 Genomic DNA Translation: AAP90975.1
AY339552 Genomic DNA Translation: AAP90988.1
AY339553 Genomic DNA Translation: AAP91001.1
AY339554 Genomic DNA Translation: AAP91014.1
AY339555 Genomic DNA Translation: AAP91027.1
AY339556 Genomic DNA Translation: AAP91040.1
AY339557 Genomic DNA Translation: AAP91053.1
AY339558 Genomic DNA Translation: AAP91066.1
AY339559 Genomic DNA Translation: AAP91079.1
AY339560 Genomic DNA Translation: AAP91092.1
AY339563 Genomic DNA Translation: AAP91131.1
AY339564 Genomic DNA Translation: AAP91144.1
AY339566 Genomic DNA Translation: AAP91170.1
AY339567 Genomic DNA Translation: AAP91183.1
AY339568 Genomic DNA Translation: AAP91196.1
AY339569 Genomic DNA Translation: AAP91209.1
AY339570 Genomic DNA Translation: AAP91222.1
AY339571 Genomic DNA Translation: AAP91235.1
AY339572 Genomic DNA Translation: AAP91248.1
AY339573 Genomic DNA Translation: AAP91261.1
AY339574 Genomic DNA Translation: AAP91274.1
AY339575 Genomic DNA Translation: AAP91287.1
AY339576 Genomic DNA Translation: AAP91300.1
AY339577 Genomic DNA Translation: AAP91313.1
AY339578 Genomic DNA Translation: AAP91326.1
AY339579 Genomic DNA Translation: AAP91339.1
AY339580 Genomic DNA Translation: AAP91352.1
AY339581 Genomic DNA Translation: AAP91365.1
AY339582 Genomic DNA Translation: AAP91378.1
AY339583 Genomic DNA Translation: AAP91391.1
AY339584 Genomic DNA Translation: AAP91404.1
AY339585 Genomic DNA Translation: AAP91417.1
AY339586 Genomic DNA Translation: AAP91430.1
AY339587 Genomic DNA Translation: AAP91443.1
AY339588 Genomic DNA Translation: AAP91456.1
AY339589 Genomic DNA Translation: AAP91469.1
AY339590 Genomic DNA Translation: AAP91482.1
AY339591 Genomic DNA Translation: AAP91495.1
AY339592 Genomic DNA Translation: AAP91508.1
AY339593 Genomic DNA Translation: AAP91521.1
AF346963 Genomic DNA Translation: AAK17209.1
AF346964 Genomic DNA Translation: AAK17222.1
AF346965 Genomic DNA Translation: AAK17235.1
AF346966 Genomic DNA Translation: AAK17248.1
AF346967 Genomic DNA Translation: AAK17261.1
AF346970 Genomic DNA Translation: AAK17300.1
AF346971 Genomic DNA Translation: AAK17313.1
AF346972 Genomic DNA Translation: AAK17326.1
AF346973 Genomic DNA Translation: AAK17339.1
AF346974 Genomic DNA Translation: AAK17352.1
AF346975 Genomic DNA Translation: AAK17365.1
AF346976 Genomic DNA Translation: AAK17378.1
AF346977 Genomic DNA Translation: AAK17391.1
AF346978 Genomic DNA Translation: AAK17404.1
AF346979 Genomic DNA Translation: AAK17417.1
AF346980 Genomic DNA Translation: AAK17430.1
AF346981 Genomic DNA Translation: AAK17443.1
AF346982 Genomic DNA Translation: AAK17456.1
AF346983 Genomic DNA Translation: AAK17469.1
AF346984 Genomic DNA Translation: AAK17482.1
AF346988 Genomic DNA Translation: AAK17534.1
AF346989 Genomic DNA Translation: AAK17547.1
AF346990 Genomic DNA Translation: AAK17560.1
AF346991 Genomic DNA Translation: AAK17573.1
AF346993 Genomic DNA Translation: AAK17599.1
AF346994 Genomic DNA Translation: AAK17612.1
AF346995 Genomic DNA Translation: AAK17625.1
AF346998 Genomic DNA Translation: AAK17664.1
AF347000 Genomic DNA Translation: AAK17690.1
AF347001 Genomic DNA Translation: AAK17703.1
AF347003 Genomic DNA Translation: AAK17729.1
AF347004 Genomic DNA Translation: AAK17742.1
AF347007 Genomic DNA Translation: AAK17781.1
AF347010 Genomic DNA Translation: AAK17820.1
AF347011 Genomic DNA Translation: AAK17833.1
AF347012 Genomic DNA Translation: AAK17846.1
AF347013 Genomic DNA Translation: AAK17859.1
AF347014 Genomic DNA Translation: AAK17872.1
AF347015 Genomic DNA Translation: AAK17885.1
AY289051 Genomic DNA Translation: AAP47882.1
AY289052 Genomic DNA Translation: AAP47895.1
AY289053 Genomic DNA Translation: AAP47908.1
AY289055 Genomic DNA Translation: AAP47934.1
AY289056 Genomic DNA Translation: AAP47947.1
AY289057 Genomic DNA Translation: AAP47960.1
AY289058 Genomic DNA Translation: AAP47973.1
AY289059 Genomic DNA Translation: AAP47986.1
AY289060 Genomic DNA Translation: AAP47999.1
AY289061 Genomic DNA Translation: AAP48012.1
AY289062 Genomic DNA Translation: AAP48025.1
AY289064 Genomic DNA Translation: AAP48051.1
AY289065 Genomic DNA Translation: AAP48064.1
AY289066 Genomic DNA Translation: AAP48077.1
AY289067 Genomic DNA Translation: AAP48090.1
AY289068 Genomic DNA Translation: AAP48103.1
AY289069 Genomic DNA Translation: AAP48116.1
AY289070 Genomic DNA Translation: AAP48129.1
AY289071 Genomic DNA Translation: AAP48142.1
AY289073 Genomic DNA Translation: AAP48168.1
AY289074 Genomic DNA Translation: AAP48181.1
AY289075 Genomic DNA Translation: AAP48194.1
AY289076 Genomic DNA Translation: AAP48207.1
AY289077 Genomic DNA Translation: AAP48220.1
AY289078 Genomic DNA Translation: AAP48233.1
AY289079 Genomic DNA Translation: AAP48246.1
AY289080 Genomic DNA Translation: AAP48259.1
AY289081 Genomic DNA Translation: AAP48272.1
AY289082 Genomic DNA Translation: AAP48285.1
AY289083 Genomic DNA Translation: AAP48298.1
AY289084 Genomic DNA Translation: AAP48311.1
AY289085 Genomic DNA Translation: AAP48324.1
AY289086 Genomic DNA Translation: AAP48337.1
AY289088 Genomic DNA Translation: AAP48363.1
AY289089 Genomic DNA Translation: AAP48376.1
AY289090 Genomic DNA Translation: AAP48389.1
AY289092 Genomic DNA Translation: AAP48415.1
AY289093 Genomic DNA Translation: AAP48427.1
AY289094 Genomic DNA Translation: AAP48440.1
AY289095 Genomic DNA Translation: AAP48453.1
AY289096 Genomic DNA Translation: AAP48466.1
AY289097 Genomic DNA Translation: AAP48479.1
AY289098 Genomic DNA Translation: AAP48492.1
AY289099 Genomic DNA Translation: AAP48505.1
AY289100 Genomic DNA Translation: AAP48518.1
AY289101 Genomic DNA Translation: AAP48531.1
AY289102 Genomic DNA Translation: AAP48544.1
AY495090 Genomic DNA Translation: AAR92498.1
AY495091 Genomic DNA Translation: AAR92511.1
AY495092 Genomic DNA Translation: AAR92524.1
AY495093 Genomic DNA Translation: AAR92537.1
AY495094 Genomic DNA Translation: AAR92550.1
AY495095 Genomic DNA Translation: AAR92563.1
AY495096 Genomic DNA Translation: AAR92576.1
AY495097 Genomic DNA Translation: AAR92589.1
AY495098 Genomic DNA Translation: AAR92602.1
AY495099 Genomic DNA Translation: AAR92615.1
AY495100 Genomic DNA Translation: AAR92628.1
AY495101 Genomic DNA Translation: AAR92641.1
AY495102 Genomic DNA Translation: AAR92654.1
AY495103 Genomic DNA Translation: AAR92667.1
AY495104 Genomic DNA Translation: AAR92680.1
AY495105 Genomic DNA Translation: AAR92693.1
AY495106 Genomic DNA Translation: AAR92706.1
AY495107 Genomic DNA Translation: AAR92719.1
AY495108 Genomic DNA Translation: AAR92732.1
AY495109 Genomic DNA Translation: AAR92745.1
AY495110 Genomic DNA Translation: AAR92758.1
AY495111 Genomic DNA Translation: AAR92771.1
AY495112 Genomic DNA Translation: AAR92784.1
AY495113 Genomic DNA Translation: AAR92797.1
AY495114 Genomic DNA Translation: AAR92810.1
AY495116 Genomic DNA Translation: AAR92836.1
AY495117 Genomic DNA Translation: AAR92849.1
AY495118 Genomic DNA Translation: AAR92862.1
AY495119 Genomic DNA Translation: AAR92875.1
AY495120 Genomic DNA Translation: AAR92888.1
AY495121 Genomic DNA Translation: AAR92901.1
AY495122 Genomic DNA Translation: AAR92914.1
AY495123 Genomic DNA Translation: AAR92927.1
AY495124 Genomic DNA Translation: AAR92940.1
AY495125 Genomic DNA Translation: AAR92953.1
AY495126 Genomic DNA Translation: AAR92966.1
AY495127 Genomic DNA Translation: AAR92979.1
AY495128 Genomic DNA Translation: AAR92992.1
AY495129 Genomic DNA Translation: AAR93005.1
AY495130 Genomic DNA Translation: AAR93018.1
AY495131 Genomic DNA Translation: AAR93031.1
AY495132 Genomic DNA Translation: AAR93044.1
AY495133 Genomic DNA Translation: AAR93057.1
AY495134 Genomic DNA Translation: AAR93070.1
AY495135 Genomic DNA Translation: AAR93083.1
AY495136 Genomic DNA Translation: AAR93096.1
AY495137 Genomic DNA Translation: AAR93109.1
AY495138 Genomic DNA Translation: AAR93122.1
AY495139 Genomic DNA Translation: AAR93135.1
AY495140 Genomic DNA Translation: AAR93148.1
AY495141 Genomic DNA Translation: AAR93161.1
AY495142 Genomic DNA Translation: AAR93174.1
AY495143 Genomic DNA Translation: AAR93187.1
AY495144 Genomic DNA Translation: AAR93200.1
AY495145 Genomic DNA Translation: AAR93213.1
AY495147 Genomic DNA Translation: AAR93239.1
AY495148 Genomic DNA Translation: AAR93252.1
AY495149 Genomic DNA Translation: AAR93265.1
AY495150 Genomic DNA Translation: AAR93278.1
AY495151 Genomic DNA Translation: AAR93291.1
AY495152 Genomic DNA Translation: AAR93304.1
AY495153 Genomic DNA Translation: AAR93317.1
AY495154 Genomic DNA Translation: AAR93330.1
AY495155 Genomic DNA Translation: AAR93343.1
AY495156 Genomic DNA Translation: AAR93356.1
AY495157 Genomic DNA Translation: AAR93369.1
AY495158 Genomic DNA Translation: AAR93382.1
AY495159 Genomic DNA Translation: AAR93395.1
AY495160 Genomic DNA Translation: AAR93408.1
AY495161 Genomic DNA Translation: AAR93421.1
AY495162 Genomic DNA Translation: AAR93434.1
AY495163 Genomic DNA Translation: AAR93447.1
AY495164 Genomic DNA Translation: AAR93460.1
AY495165 Genomic DNA Translation: AAR93473.1
AY495166 Genomic DNA Translation: AAR93486.1
AY495167 Genomic DNA Translation: AAR93499.1
AY495168 Genomic DNA Translation: AAR93512.1
AY495169 Genomic DNA Translation: AAR93525.1
AY495170 Genomic DNA Translation: AAR93538.1
AY495171 Genomic DNA Translation: AAR93551.1
AY495172 Genomic DNA Translation: AAR93564.1
AY495173 Genomic DNA Translation: AAR93577.1
AY495174 Genomic DNA Translation: AAR93590.1
AY495175 Genomic DNA Translation: AAR93603.1
AY495176 Genomic DNA Translation: AAR93616.1
AY495177 Genomic DNA Translation: AAR93629.1
AY495178 Genomic DNA Translation: AAR93642.1
AY495179 Genomic DNA Translation: AAR93655.1
AY495180 Genomic DNA Translation: AAR93668.1
AY495181 Genomic DNA Translation: AAR93681.1
AY495182 Genomic DNA Translation: AAR93694.1
AY495183 Genomic DNA Translation: AAR93707.1
AY495184 Genomic DNA Translation: AAR93720.1
AY495185 Genomic DNA Translation: AAR93733.1
AY495186 Genomic DNA Translation: AAR93746.1
AY495187 Genomic DNA Translation: AAR93759.1
AY495188 Genomic DNA Translation: AAR93772.1
AY495189 Genomic DNA Translation: AAR93785.1
AY495190 Genomic DNA Translation: AAR93798.1
AY495191 Genomic DNA Translation: AAR93811.1
AY495192 Genomic DNA Translation: AAR93824.1
AY495193 Genomic DNA Translation: AAR93837.1
AY495194 Genomic DNA Translation: AAR93850.1
AY495195 Genomic DNA Translation: AAR93863.1
AY495196 Genomic DNA Translation: AAR93876.1
AY495197 Genomic DNA Translation: AAR93889.1
AY495198 Genomic DNA Translation: AAR93902.1
AY495199 Genomic DNA Translation: AAR93915.1
AY495200 Genomic DNA Translation: AAR93928.1
AY495201 Genomic DNA Translation: AAR93941.1
AY495202 Genomic DNA Translation: AAR93954.1
AY495203 Genomic DNA Translation: AAR93967.1
AY495204 Genomic DNA Translation: AAR93980.1
AY495205 Genomic DNA Translation: AAR93993.1
AY495206 Genomic DNA Translation: AAR94006.1
AY495207 Genomic DNA Translation: AAR94019.1
AY495208 Genomic DNA Translation: AAR94032.1
AY495209 Genomic DNA Translation: AAR94045.1
AY495210 Genomic DNA Translation: AAR94058.1
AY495211 Genomic DNA Translation: AAR94071.1
AY495212 Genomic DNA Translation: AAR94084.1
AY495213 Genomic DNA Translation: AAR94097.1
AY495214 Genomic DNA Translation: AAR94110.1
AY495215 Genomic DNA Translation: AAR94123.1
AY495216 Genomic DNA Translation: AAR94136.1
AY495217 Genomic DNA Translation: AAR94149.1
AY495218 Genomic DNA Translation: AAR94162.1
AY495219 Genomic DNA Translation: AAR94175.1
AY495220 Genomic DNA Translation: AAR94188.1
AY495221 Genomic DNA Translation: AAR94201.1
AY495222 Genomic DNA Translation: AAR94214.1
AY495223 Genomic DNA Translation: AAR94227.1
AY495224 Genomic DNA Translation: AAR94240.1
AY495225 Genomic DNA Translation: AAR94253.1
AY495226 Genomic DNA Translation: AAR94266.1
AY495228 Genomic DNA Translation: AAR94292.1
AY495230 Genomic DNA Translation: AAR94318.1
AY495231 Genomic DNA Translation: AAR94331.1
AY495232 Genomic DNA Translation: AAR94344.1
AY495233 Genomic DNA Translation: AAR94357.1
AY495234 Genomic DNA Translation: AAR94370.1
AY495235 Genomic DNA Translation: AAR94383.1
AY495236 Genomic DNA Translation: AAR94396.1
AY495237 Genomic DNA Translation: AAR94409.1
AY495239 Genomic DNA Translation: AAR94435.1
AY495240 Genomic DNA Translation: AAR94448.1
AY495241 Genomic DNA Translation: AAR94461.1
AY495242 Genomic DNA Translation: AAR94474.1
AY495243 Genomic DNA Translation: AAR94487.1
AY495244 Genomic DNA Translation: AAR94500.1
AY495245 Genomic DNA Translation: AAR94513.1
AY495246 Genomic DNA Translation: AAR94526.1
AY495247 Genomic DNA Translation: AAR94539.1
AY495248 Genomic DNA Translation: AAR94552.1
AY495249 Genomic DNA Translation: AAR94565.1
AY495250 Genomic DNA Translation: AAR94578.1
AY495251 Genomic DNA Translation: AAR94591.1
AY495252 Genomic DNA Translation: AAR94604.1
AY495253 Genomic DNA Translation: AAR94617.1
AY495254 Genomic DNA Translation: AAR94630.1
AY495255 Genomic DNA Translation: AAR94643.1
AY495256 Genomic DNA Translation: AAR94656.1
AY495258 Genomic DNA Translation: AAR94682.1
AY495259 Genomic DNA Translation: AAR94695.1
AY495261 Genomic DNA Translation: AAR94721.1
AY495262 Genomic DNA Translation: AAR94734.1
AY495263 Genomic DNA Translation: AAR94747.1
AY495264 Genomic DNA Translation: AAR94760.1
AY495265 Genomic DNA Translation: AAR94773.1
AY495266 Genomic DNA Translation: AAR94786.1
AY495267 Genomic DNA Translation: AAR94799.1
AY495268 Genomic DNA Translation: AAR94812.1
AY495269 Genomic DNA Translation: AAR94825.1
AY495270 Genomic DNA Translation: AAR94838.1
AY495271 Genomic DNA Translation: AAR94851.1
AY495272 Genomic DNA Translation: AAR94864.1
AY495273 Genomic DNA Translation: AAR94877.1
AY495274 Genomic DNA Translation: AAR94890.1
AY495275 Genomic DNA Translation: AAR94903.1
AY495276 Genomic DNA Translation: AAR94916.1
AY495277 Genomic DNA Translation: AAR94929.1
AY495278 Genomic DNA Translation: AAR94942.1
AY495279 Genomic DNA Translation: AAR94955.1
AY495280 Genomic DNA Translation: AAR94968.1
AY495281 Genomic DNA Translation: AAR94981.1
AY495282 Genomic DNA Translation: AAR94994.1
AY495283 Genomic DNA Translation: AAR95007.1
AY495284 Genomic DNA Translation: AAR95020.1
AY495285 Genomic DNA Translation: AAR95033.1
AY495286 Genomic DNA Translation: AAR95046.1
AY495287 Genomic DNA Translation: AAR95059.1
AY495288 Genomic DNA Translation: AAR95072.1
AY495289 Genomic DNA Translation: AAR95085.1
AY495290 Genomic DNA Translation: AAR95098.1
AY495291 Genomic DNA Translation: AAR95111.1
AY495292 Genomic DNA Translation: AAR95124.1
AY495293 Genomic DNA Translation: AAR95137.1
AY495294 Genomic DNA Translation: AAR95150.1
AY495295 Genomic DNA Translation: AAR95163.1
AY495297 Genomic DNA Translation: AAR95189.1
AY495298 Genomic DNA Translation: AAR95202.1
AY495299 Genomic DNA Translation: AAR95215.1
AY495300 Genomic DNA Translation: AAR95228.1
AY495301 Genomic DNA Translation: AAR95241.1
AY495302 Genomic DNA Translation: AAR95254.1
AY495303 Genomic DNA Translation: AAR95267.1
AY495304 Genomic DNA Translation: AAR95280.1
AY495305 Genomic DNA Translation: AAR95293.1
AY495306 Genomic DNA Translation: AAR95306.1
AY495307 Genomic DNA Translation: AAR95319.1
AY495308 Genomic DNA Translation: AAR95332.1
AY495309 Genomic DNA Translation: AAR95345.1
AY495310 Genomic DNA Translation: AAR95358.1
AY495311 Genomic DNA Translation: AAR95371.1
AY495312 Genomic DNA Translation: AAR95384.1
AY495313 Genomic DNA Translation: AAR95397.1
AY495314 Genomic DNA Translation: AAR95410.1
AY495315 Genomic DNA Translation: AAR95423.1
AY495316 Genomic DNA Translation: AAR95436.1
AY495317 Genomic DNA Translation: AAR95449.1
AY495318 Genomic DNA Translation: AAR95462.1
AY495319 Genomic DNA Translation: AAR95475.1
AY495320 Genomic DNA Translation: AAR95488.1
AY495322 Genomic DNA Translation: AAR95514.1
AY495323 Genomic DNA Translation: AAR95527.1
AY495324 Genomic DNA Translation: AAR95540.1
AY495325 Genomic DNA Translation: AAR95553.1
AY495326 Genomic DNA Translation: AAR95566.1
AY495327 Genomic DNA Translation: AAR95579.1
AY495328 Genomic DNA Translation: AAR95592.1
AY495329 Genomic DNA Translation: AAR95605.1
AY495330 Genomic DNA Translation: AAR95618.1
M10546 Genomic DNA Translation: AAA65503.1
PIRiA00463 ODHU1
RefSeqiYP_003024028.1, NC_012920.1

Genome annotation databases

EnsembliENST00000361624; ENSP00000354499; ENSG00000198804
GeneIDi4512
KEGGihsa:4512

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiCOX1_HUMAN
AccessioniPrimary (citable) accession number: P00395
Secondary accession number(s): Q34770
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: July 21, 1986
Last modified: May 23, 2018
This is version 193 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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