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Protein

Cytochrome c oxidase subunit 3

Gene

MT-CO3

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

Functioni

Subunits I, II and III form the functional core of the enzyme complex.

GO - Molecular functioni

GO - Biological processi

  • aerobic respiration Source: GO_Central
  • mitochondrial electron transport, cytochrome c to oxygen Source: GO_Central
  • respiratory chain complex IV assembly Source: UniProtKB

Enzyme and pathway databases

ReactomeiR-HSA-5628897 TP53 Regulates Metabolic Genes
R-HSA-611105 Respiratory electron transport

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 3
Alternative name(s):
Cytochrome c oxidase polypeptide III
Gene namesi
Name:MT-CO3
Synonyms:COIII, COXIII, MTCO3
Encoded oniMitochondrion
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Mitochondrion

Organism-specific databases

EuPathDBiHostDB:ENSG00000198938.2
HGNCiHGNC:7422 MT-CO3
MIMi516050 gene
neXtProtiNX_P00414

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
Transmembranei15 – 35HelicalSequence analysisAdd BLAST21
Transmembranei42 – 59HelicalSequence analysisAdd BLAST18
Transmembranei81 – 101HelicalSequence analysisAdd BLAST21
Transmembranei127 – 147HelicalSequence analysisAdd BLAST21
Transmembranei159 – 179HelicalSequence analysisAdd BLAST21
Transmembranei197 – 217HelicalSequence analysisAdd BLAST21
Transmembranei239 – 259HelicalSequence analysisAdd BLAST21

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_00216778G → S in LHON; secondary mutation; does not seem to directly cause the disease. 1 PublicationCorresponds to variant dbSNP:rs267606611Ensembl.1
Natural variantiVAR_002168200A → T in LHON; possible rare primary mutation. 1 PublicationCorresponds to variant dbSNP:rs200613617Ensembl.1
Mitochondrial complex IV deficiency (MT-C4D)1 Publication
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_03305794 – 98Missing in MT-C4D; with RM-MT. 1 Publication5
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

Keywords - Diseasei

Disease mutation, Leber hereditary optic neuropathy, Primary mitochondrial disease

Organism-specific databases

DisGeNETi4514
GeneReviewsiMT-CO3
MalaCardsiMT-CO3
MIMi220110 phenotype
535000 phenotype
550500 phenotype
OpenTargetsiENSG00000198938
Orphaneti99845 Genetic recurrent myoglobinuria
254905 Isolated cytochrome C oxidase deficiency
104 Leber hereditary optic neuropathy
550 MELAS

Chemistry databases

DrugBankiDB02659 Cholic Acid
DB04464 N-Formylmethionine

Polymorphism and mutation databases

DMDMi6648058

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001837931 – 261Cytochrome c oxidase subunit 3Add BLAST261

Proteomic databases

EPDiP00414
PaxDbiP00414
PeptideAtlasiP00414
PRIDEiP00414
ProteomicsDBi51248
TopDownProteomicsiP00414

PTM databases

iPTMnetiP00414
PhosphoSitePlusiP00414

Expressioni

Gene expression databases

BgeeiENSG00000198938 Expressed in 89 organ(s), highest expression level in left lobe of thyroid gland
ExpressionAtlasiP00414 baseline and differential
GenevisibleiP00414 HS

Organism-specific databases

HPAiHPA042788

Interactioni

Binary interactionsi

WithEntry#Exp.IntActNotes
SNCAP378403EBI-3932264,EBI-985879

Protein-protein interaction databases

BioGridi110617, 8 interactors
CORUMiP00414
IntActiP00414, 9 interactors
MINTiP00414
STRINGi9606.ENSP00000354982

Structurei

3D structure databases

ProteinModelPortaliP00414
SMRiP00414
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG4664 Eukaryota
COG1845 LUCA
GeneTreeiENSGT00390000013064
HOGENOMiHOG000264954
HOVERGENiHBG016686
InParanoidiP00414
KOiK02262
OMAiSIYWWGS
OrthoDBiEOG091G0GH8
PhylomeDBiP00414
TreeFamiTF343435

Family and domain databases

CDDicd01665 Cyt_c_Oxidase_III, 1 hit
Gene3Di1.20.120.80, 1 hit
InterProiView protein in InterPro
IPR024791 Cyt_c/ubiquinol_Oxase_su3
IPR033945 Cyt_c_oxase_su3_dom
IPR000298 Cyt_c_oxidase-like_su3
IPR035973 Cyt_c_oxidase_su3-like_sf
IPR013833 Cyt_c_oxidase_su3_a-hlx
PANTHERiPTHR11403 PTHR11403, 1 hit
PfamiView protein in Pfam
PF00510 COX3, 1 hit
SUPFAMiSSF81452 SSF81452, 1 hit
PROSITEiView protein in PROSITE
PS50253 COX3, 1 hit

Sequencei

Sequence statusi: Complete.

P00414-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MTHQSHAYHM VKPSPWPLTG ALSALLMTSG LAMWFHFHSM TLLMLGLLTN
60 70 80 90 100
TLTMYQWWRD VTRESTYQGH HTPPVQKGLR YGMILFITSE VFFFAGFFWA
110 120 130 140 150
FYHSSLAPTP QLGGHWPPTG ITPLNPLEVP LLNTSVLLAS GVSITWAHHS
160 170 180 190 200
LMENNRNQMI QALLITILLG LYFTLLQASE YFESPFTISD GIYGSTFFVA
210 220 230 240 250
TGFHGLHVII GSTFLTICFI RQLMFHFTSK HHFGFEAAAW YWHFVDVVWL
260
FLYVSIYWWG S
Length:261
Mass (Da):29,951
Last modified:May 30, 2000 - v2
Checksum:iA04385EF748B7C14
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti118P → R in CAA24032 (PubMed:7219534).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0085733H → R1 Publication1
Natural variantiVAR_00857435F → S1 Publication1
Natural variantiVAR_00216778G → S in LHON; secondary mutation; does not seem to directly cause the disease. 1 PublicationCorresponds to variant dbSNP:rs267606611Ensembl.1
Natural variantiVAR_00857591V → I1 PublicationCorresponds to variant dbSNP:rs2853825Ensembl.1
Natural variantiVAR_03305794 – 98Missing in MT-C4D; with RM-MT. 1 Publication5
Natural variantiVAR_008576177Q → R1 Publication1
Natural variantiVAR_002168200A → T in LHON; possible rare primary mutation. 1 PublicationCorresponds to variant dbSNP:rs200613617Ensembl.1
Natural variantiVAR_002169251F → L Found in two patients with a diagnosis of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome; unknown pathological significance. 2 Publications1
Natural variantiVAR_008577254V → I1 PublicationCorresponds to variant dbSNP:rs200809063Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J01415 Genomic DNA Translation: AAB58949.2
V00662 Genomic DNA Translation: CAA24032.1
DQ654394 Genomic DNA Translation: ABG27956.1
DQ654395 Genomic DNA Translation: ABG27958.1
DQ654396 Genomic DNA Translation: ABG27960.1
DQ654397 Genomic DNA Translation: ABG27962.1
DQ654398 Genomic DNA Translation: ABG27964.1
DQ654399 Genomic DNA Translation: ABG27966.1
DQ654400 Genomic DNA Translation: ABG27968.1
DQ654401 Genomic DNA Translation: ABG27970.1
DQ654402 Genomic DNA Translation: ABG27972.1
DQ654403 Genomic DNA Translation: ABG27974.1
DQ654404 Genomic DNA Translation: ABG27976.1
DQ654405 Genomic DNA Translation: ABG27978.1
DQ654406 Genomic DNA Translation: ABG27980.1
DQ654407 Genomic DNA Translation: ABG27982.1
DQ654408 Genomic DNA Translation: ABG27984.1
DQ654409 Genomic DNA Translation: ABG27986.1
DQ654410 Genomic DNA Translation: ABG27988.1
DQ654411 Genomic DNA Translation: ABG27990.1
DQ654412 Genomic DNA Translation: ABG27992.1
DQ654413 Genomic DNA Translation: ABG27994.1
DQ654414 Genomic DNA Translation: ABG27996.1
DQ654415 Genomic DNA Translation: ABG27998.1
DQ654416 Genomic DNA Translation: ABG28000.1
DQ654417 Genomic DNA Translation: ABG28002.1
DQ654418 Genomic DNA Translation: ABG28004.1
DQ654419 Genomic DNA Translation: ABG28006.1
DQ654420 Genomic DNA Translation: ABG28008.1
DQ654421 Genomic DNA Translation: ABG28010.1
DQ654422 Genomic DNA Translation: ABG28012.1
DQ654423 Genomic DNA Translation: ABG28014.1
DQ654424 Genomic DNA Translation: ABG28016.1
DQ654425 Genomic DNA Translation: ABG28018.1
DQ654426 Genomic DNA Translation: ABG28020.1
DQ654427 Genomic DNA Translation: ABG28022.1
DQ654428 Genomic DNA Translation: ABG28024.1
DQ654429 Genomic DNA Translation: ABG28026.1
DQ654430 Genomic DNA Translation: ABG28028.1
DQ654431 Genomic DNA Translation: ABG28030.1
DQ654432 Genomic DNA Translation: ABG28032.1
DQ654433 Genomic DNA Translation: ABG28034.1
DQ654434 Genomic DNA Translation: ABG28036.1
DQ654435 Genomic DNA Translation: ABG28038.1
DQ654436 Genomic DNA Translation: ABG28040.1
DQ654437 Genomic DNA Translation: ABG28042.1
DQ654438 Genomic DNA Translation: ABG28044.1
DQ654439 Genomic DNA Translation: ABG28046.1
DQ654440 Genomic DNA Translation: ABG28048.1
DQ654441 Genomic DNA Translation: ABG28050.1
DQ654442 Genomic DNA Translation: ABG28052.1
DQ654443 Genomic DNA Translation: ABG28054.1
AF004341 Genomic DNA Translation: AAB63452.1
PIRiA00482 OTHU3
RefSeqiYP_003024032.1, NC_012920.1

Genome annotation databases

EnsembliENST00000362079; ENSP00000354982; ENSG00000198938
GeneIDi4514
KEGGihsa:4514

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J01415 Genomic DNA Translation: AAB58949.2
V00662 Genomic DNA Translation: CAA24032.1
DQ654394 Genomic DNA Translation: ABG27956.1
DQ654395 Genomic DNA Translation: ABG27958.1
DQ654396 Genomic DNA Translation: ABG27960.1
DQ654397 Genomic DNA Translation: ABG27962.1
DQ654398 Genomic DNA Translation: ABG27964.1
DQ654399 Genomic DNA Translation: ABG27966.1
DQ654400 Genomic DNA Translation: ABG27968.1
DQ654401 Genomic DNA Translation: ABG27970.1
DQ654402 Genomic DNA Translation: ABG27972.1
DQ654403 Genomic DNA Translation: ABG27974.1
DQ654404 Genomic DNA Translation: ABG27976.1
DQ654405 Genomic DNA Translation: ABG27978.1
DQ654406 Genomic DNA Translation: ABG27980.1
DQ654407 Genomic DNA Translation: ABG27982.1
DQ654408 Genomic DNA Translation: ABG27984.1
DQ654409 Genomic DNA Translation: ABG27986.1
DQ654410 Genomic DNA Translation: ABG27988.1
DQ654411 Genomic DNA Translation: ABG27990.1
DQ654412 Genomic DNA Translation: ABG27992.1
DQ654413 Genomic DNA Translation: ABG27994.1
DQ654414 Genomic DNA Translation: ABG27996.1
DQ654415 Genomic DNA Translation: ABG27998.1
DQ654416 Genomic DNA Translation: ABG28000.1
DQ654417 Genomic DNA Translation: ABG28002.1
DQ654418 Genomic DNA Translation: ABG28004.1
DQ654419 Genomic DNA Translation: ABG28006.1
DQ654420 Genomic DNA Translation: ABG28008.1
DQ654421 Genomic DNA Translation: ABG28010.1
DQ654422 Genomic DNA Translation: ABG28012.1
DQ654423 Genomic DNA Translation: ABG28014.1
DQ654424 Genomic DNA Translation: ABG28016.1
DQ654425 Genomic DNA Translation: ABG28018.1
DQ654426 Genomic DNA Translation: ABG28020.1
DQ654427 Genomic DNA Translation: ABG28022.1
DQ654428 Genomic DNA Translation: ABG28024.1
DQ654429 Genomic DNA Translation: ABG28026.1
DQ654430 Genomic DNA Translation: ABG28028.1
DQ654431 Genomic DNA Translation: ABG28030.1
DQ654432 Genomic DNA Translation: ABG28032.1
DQ654433 Genomic DNA Translation: ABG28034.1
DQ654434 Genomic DNA Translation: ABG28036.1
DQ654435 Genomic DNA Translation: ABG28038.1
DQ654436 Genomic DNA Translation: ABG28040.1
DQ654437 Genomic DNA Translation: ABG28042.1
DQ654438 Genomic DNA Translation: ABG28044.1
DQ654439 Genomic DNA Translation: ABG28046.1
DQ654440 Genomic DNA Translation: ABG28048.1
DQ654441 Genomic DNA Translation: ABG28050.1
DQ654442 Genomic DNA Translation: ABG28052.1
DQ654443 Genomic DNA Translation: ABG28054.1
AF004341 Genomic DNA Translation: AAB63452.1
PIRiA00482 OTHU3
RefSeqiYP_003024032.1, NC_012920.1

3D structure databases

ProteinModelPortaliP00414
SMRiP00414
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110617, 8 interactors
CORUMiP00414
IntActiP00414, 9 interactors
MINTiP00414
STRINGi9606.ENSP00000354982

Chemistry databases

DrugBankiDB02659 Cholic Acid
DB04464 N-Formylmethionine

Protein family/group databases

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

PTM databases

iPTMnetiP00414
PhosphoSitePlusiP00414

Polymorphism and mutation databases

DMDMi6648058

Proteomic databases

EPDiP00414
PaxDbiP00414
PeptideAtlasiP00414
PRIDEiP00414
ProteomicsDBi51248
TopDownProteomicsiP00414

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000362079; ENSP00000354982; ENSG00000198938
GeneIDi4514
KEGGihsa:4514

Organism-specific databases

CTDi4514
DisGeNETi4514
EuPathDBiHostDB:ENSG00000198938.2
GeneCardsiMT-CO3
GeneReviewsiMT-CO3
H-InvDBiHIX0060139
HGNCiHGNC:7422 MT-CO3
HPAiHPA042788
MalaCardsiMT-CO3
MIMi220110 phenotype
516050 gene
535000 phenotype
550500 phenotype
neXtProtiNX_P00414
OpenTargetsiENSG00000198938
Orphaneti99845 Genetic recurrent myoglobinuria
254905 Isolated cytochrome C oxidase deficiency
104 Leber hereditary optic neuropathy
550 MELAS
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4664 Eukaryota
COG1845 LUCA
GeneTreeiENSGT00390000013064
HOGENOMiHOG000264954
HOVERGENiHBG016686
InParanoidiP00414
KOiK02262
OMAiSIYWWGS
OrthoDBiEOG091G0GH8
PhylomeDBiP00414
TreeFamiTF343435

Enzyme and pathway databases

ReactomeiR-HSA-5628897 TP53 Regulates Metabolic Genes
R-HSA-611105 Respiratory electron transport

Miscellaneous databases

ChiTaRSiCOX3 human
GeneWikiiMT-CO3
GenomeRNAii4514
PROiPR:P00414
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000198938 Expressed in 89 organ(s), highest expression level in left lobe of thyroid gland
ExpressionAtlasiP00414 baseline and differential
GenevisibleiP00414 HS

Family and domain databases

CDDicd01665 Cyt_c_Oxidase_III, 1 hit
Gene3Di1.20.120.80, 1 hit
InterProiView protein in InterPro
IPR024791 Cyt_c/ubiquinol_Oxase_su3
IPR033945 Cyt_c_oxase_su3_dom
IPR000298 Cyt_c_oxidase-like_su3
IPR035973 Cyt_c_oxidase_su3-like_sf
IPR013833 Cyt_c_oxidase_su3_a-hlx
PANTHERiPTHR11403 PTHR11403, 1 hit
PfamiView protein in Pfam
PF00510 COX3, 1 hit
SUPFAMiSSF81452 SSF81452, 1 hit
PROSITEiView protein in PROSITE
PS50253 COX3, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCOX3_HUMAN
AccessioniPrimary (citable) accession number: P00414
Secondary accession number(s): Q14Y83
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: May 30, 2000
Last modified: November 7, 2018
This is version 181 of the entry and version 2 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

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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