UniProtKB - P14854 (CX6B1_HUMAN)
Cytochrome c oxidase subunit 6B1
COX6B1
Functioni
Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.
By similarity: oxidative phosphorylation Pathwayi
This protein is involved in the pathway oxidative phosphorylation, which is part of Energy metabolism.By similarityView all proteins of this organism that are known to be involved in the pathway oxidative phosphorylation and in Energy metabolism.
GO - Molecular functioni
- cytochrome-c oxidase activity Source: UniProtKB
GO - Biological processi
- oxidative phosphorylation Source: UniProtKB-UniPathway
- substantia nigra development Source: UniProtKB
Enzyme and pathway databases
PathwayCommonsi | P14854 |
Reactomei | R-HSA-5628897, TP53 Regulates Metabolic Genes R-HSA-611105, Respiratory electron transport R-HSA-9707564, Cytoprotection by HMOX1 |
SignaLinki | P14854 |
UniPathwayi | UPA00705 |
Names & Taxonomyi
Protein namesi | Recommended name: Cytochrome c oxidase subunit 6B1Alternative name(s): Cytochrome c oxidase subunit VIb isoform 1 Short name: COX VIb-1 |
Gene namesi | Name:COX6B1 Synonyms:COX6B |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:2280, COX6B1 |
MIMi | 124089, gene |
neXtProti | NX_P14854 |
VEuPathDBi | HostDB:ENSG00000126267 |
Subcellular locationi
Mitochondrion
- Mitochondrion inner membrane 1 Publication; Peripheral membrane protein 1 Publication; Intermembrane side 1 Publication
Mitochondrion
- mitochondrial inner membrane Source: Reactome
- mitochondrial membrane Source: ComplexPortal
- mitochondrial respiratory chain complex IV Source: ComplexPortal
- mitochondrion Source: HPA
Other locations
- respiratory chain complex IV Source: GO_Central
Keywords - Cellular componenti
Membrane, Mitochondrion, Mitochondrion inner membranePathology & Biotechi
Involvement in diseasei
Mitochondrial complex IV deficiency, nuclear type 7 (MC4DN7)2 Publications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_084178 | 20 | R → C in MC4DN7; severely reduced COX6B1 protein levels in patient muscle. 1 Publication | 1 | |
Natural variantiVAR_046775 | 20 | R → H in MC4DN7. 1 PublicationCorresponds to variant dbSNP:rs121909602EnsemblClinVar. | 1 |
Keywords - Diseasei
Disease variant, Primary mitochondrial diseaseOrganism-specific databases
DisGeNETi | 1340 |
MalaCardsi | COX6B1 |
MIMi | 619051, phenotype |
OpenTargetsi | ENSG00000126267 |
Orphaneti | 254905, Isolated cytochrome C oxidase deficiency |
PharmGKBi | PA26797 |
Miscellaneous databases
Pharosi | P14854, Tbio |
Chemistry databases
DrugBanki | DB02659, Cholic Acid DB04464, N-Formylmethionine |
Genetic variation databases
BioMutai | COX6B1 |
DMDMi | 117115 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Initiator methioninei | RemovedCombined sources | |||
ChainiPRO_0000194912 | 2 – 86 | Cytochrome c oxidase subunit 6B1Add BLAST | 85 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 2 | N-acetylalanineCombined sources | 1 | |
Disulfide bondi | 30 ↔ 65 | PROSITE-ProRule annotation | ||
Disulfide bondi | 40 ↔ 54 | PROSITE-ProRule annotation |
Keywords - PTMi
Acetylation, Disulfide bondProteomic databases
EPDi | P14854 |
jPOSTi | P14854 |
MassIVEi | P14854 |
MaxQBi | P14854 |
PaxDbi | P14854 |
PeptideAtlasi | P14854 |
PRIDEi | P14854 |
ProteomicsDBi | 53084 |
TopDownProteomicsi | P14854 |
PTM databases
GlyGeni | P14854, 1 site, 1 O-linked glycan (1 site) |
iPTMneti | P14854 |
PhosphoSitePlusi | P14854 |
Expressioni
Gene expression databases
Bgeei | ENSG00000126267, Expressed in apex of heart and 252 other tissues |
ExpressionAtlasi | P14854, baseline and differential |
Genevisiblei | P14854, HS |
Organism-specific databases
HPAi | ENSG00000126267, Tissue enhanced (tongue) |
Interactioni
Subunit structurei
Component of the cytochrome c oxidase (complex IV, CIV), a multisubunit enzyme composed of 14 subunits. The complex is composed of a catalytic core of 3 subunits MT-CO1, MT-CO2 and MT-CO3, encoded in the mitochondrial DNA, and 11 supernumerary subunits COX4I1 (or COX4I2), COX5A, COX5B, COX6A1 (or COX6A2), COX6B1 (or COX6B2), COX6C, COX7A2 (or COX7A1), COX7B, COX7C, COX8A and NDUFA4, which are encoded in the nuclear genome (PubMed:30030519). The complex exists as a monomer or a dimer and forms supercomplexes (SCs) in the inner mitochondrial membrane with NADH-ubiquinone oxidoreductase (complex I, CI) and ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII), resulting in different assemblies (supercomplex SCI1III2IV1 and megacomplex MCI2III2IV2) (PubMed:28844695).
2 PublicationsProtein-protein interaction databases
BioGRIDi | 107733, 91 interactors |
ComplexPortali | CPX-6123, Mitochondrial respiratory chain complex IV |
IntActi | P14854, 64 interactors |
MINTi | P14854 |
STRINGi | 9606.ENSP00000246554 |
Miscellaneous databases
RNActi | P14854, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 27 – 73 | CHCHPROSITE-ProRule annotationAdd BLAST | 47 |
Motif
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Motifi | 30 – 40 | Cx9C motifPROSITE-ProRule annotationAdd BLAST | 11 | |
Motifi | 54 – 65 | Cx10C motifPROSITE-ProRule annotationAdd BLAST | 12 |
Sequence similaritiesi
Phylogenomic databases
eggNOGi | KOG3057, Eukaryota |
GeneTreei | ENSGT00940000156204 |
HOGENOMi | CLU_133964_3_1_1 |
InParanoidi | P14854 |
OMAi | HCFQNYT |
OrthoDBi | 1586678at2759 |
PhylomeDBi | P14854 |
TreeFami | TF105065 |
Family and domain databases
CDDi | cd00926, Cyt_c_Oxidase_VIb, 1 hit |
Gene3Di | 1.10.10.140, 1 hit |
InterProi | View protein in InterPro IPR003213, Cyt_c_oxidase_su6B IPR036549, Cyt_c_oxidase_su6B_sf |
PANTHERi | PTHR11387, PTHR11387, 1 hit |
Pfami | View protein in Pfam PF02297, COX6B, 1 hit |
PIRSFi | PIRSF000278, Cyt_c_oxidase_6B, 1 hit |
SUPFAMi | SSF47694, SSF47694, 1 hit |
PROSITEi | View protein in PROSITE PS51808, CHCH, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All
10 20 30 40 50
MAEDMETKIK NYKTAPFDSR FPNQNQTRNC WQNYLDFHRC QKAMTAKGGD
60 70 80
ISVCEWYQRV YQSLCPTSWV TDWDEQRAEG TFPGKI
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketK7EQD3 | K7EQD3_HUMAN | Cytochrome c oxidase subunit 6B1 | COX6B1 | 40 | Annotation score: | ||
A0A494C160 | A0A494C160_HUMAN | Cytochrome c oxidase subunit 6B1 | COX6B1 | 54 | Annotation score: |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_084178 | 20 | R → C in MC4DN7; severely reduced COX6B1 protein levels in patient muscle. 1 Publication | 1 | |
Natural variantiVAR_046775 | 20 | R → H in MC4DN7. 1 PublicationCorresponds to variant dbSNP:rs121909602EnsemblClinVar. | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X13923 mRNA Translation: CAA32114.1 X54473 mRNA Translation: CAA38352.1 AK312140 mRNA Translation: BAG35076.1 BT006945 mRNA Translation: AAP35591.1 CR456789 mRNA Translation: CAG33070.1 CR542137 mRNA Translation: CAG46934.1 AC002115 Genomic DNA Translation: AAB57628.1 BC001015 mRNA Translation: AAH01015.1 BC002478 mRNA Translation: AAH02478.1 X58139 Genomic DNA Translation: CAA41147.1 |
CCDSi | CCDS12469.1 |
PIRi | S03287, OGHU6B |
RefSeqi | NP_001854.1, NM_001863.4 |
Genome annotation databases
Ensembli | ENST00000392201; ENSP00000376037; ENSG00000126267 ENST00000592141; ENSP00000466818; ENSG00000126267 ENST00000649813; ENSP00000497926; ENSG00000126267 |
GeneIDi | 1340 |
KEGGi | hsa:1340 |
MANE-Selecti | ENST00000649813.2; ENSP00000497926.1; NM_001863.5; NP_001854.1 |
UCSCi | uc002oav.4, human |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X13923 mRNA Translation: CAA32114.1 X54473 mRNA Translation: CAA38352.1 AK312140 mRNA Translation: BAG35076.1 BT006945 mRNA Translation: AAP35591.1 CR456789 mRNA Translation: CAG33070.1 CR542137 mRNA Translation: CAG46934.1 AC002115 Genomic DNA Translation: AAB57628.1 BC001015 mRNA Translation: AAH01015.1 BC002478 mRNA Translation: AAH02478.1 X58139 Genomic DNA Translation: CAA41147.1 |
CCDSi | CCDS12469.1 |
PIRi | S03287, OGHU6B |
RefSeqi | NP_001854.1, NM_001863.4 |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
5Z62 | electron microscopy | 3.60 | H | 5-86 | [»] | |
SMRi | P14854 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 107733, 91 interactors |
ComplexPortali | CPX-6123, Mitochondrial respiratory chain complex IV |
IntActi | P14854, 64 interactors |
MINTi | P14854 |
STRINGi | 9606.ENSP00000246554 |
Chemistry databases
DrugBanki | DB02659, Cholic Acid DB04464, N-Formylmethionine |
PTM databases
GlyGeni | P14854, 1 site, 1 O-linked glycan (1 site) |
iPTMneti | P14854 |
PhosphoSitePlusi | P14854 |
Genetic variation databases
BioMutai | COX6B1 |
DMDMi | 117115 |
Proteomic databases
EPDi | P14854 |
jPOSTi | P14854 |
MassIVEi | P14854 |
MaxQBi | P14854 |
PaxDbi | P14854 |
PeptideAtlasi | P14854 |
PRIDEi | P14854 |
ProteomicsDBi | 53084 |
TopDownProteomicsi | P14854 |
Protocols and materials databases
Antibodypediai | 1083, 314 antibodies from 32 providers |
DNASUi | 1340 |
Genome annotation databases
Ensembli | ENST00000392201; ENSP00000376037; ENSG00000126267 ENST00000592141; ENSP00000466818; ENSG00000126267 ENST00000649813; ENSP00000497926; ENSG00000126267 |
GeneIDi | 1340 |
KEGGi | hsa:1340 |
MANE-Selecti | ENST00000649813.2; ENSP00000497926.1; NM_001863.5; NP_001854.1 |
UCSCi | uc002oav.4, human |
Organism-specific databases
CTDi | 1340 |
DisGeNETi | 1340 |
GeneCardsi | COX6B1 |
HGNCi | HGNC:2280, COX6B1 |
HPAi | ENSG00000126267, Tissue enhanced (tongue) |
MalaCardsi | COX6B1 |
MIMi | 124089, gene 619051, phenotype |
neXtProti | NX_P14854 |
OpenTargetsi | ENSG00000126267 |
Orphaneti | 254905, Isolated cytochrome C oxidase deficiency |
PharmGKBi | PA26797 |
VEuPathDBi | HostDB:ENSG00000126267 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG3057, Eukaryota |
GeneTreei | ENSGT00940000156204 |
HOGENOMi | CLU_133964_3_1_1 |
InParanoidi | P14854 |
OMAi | HCFQNYT |
OrthoDBi | 1586678at2759 |
PhylomeDBi | P14854 |
TreeFami | TF105065 |
Enzyme and pathway databases
UniPathwayi | UPA00705 |
PathwayCommonsi | P14854 |
Reactomei | R-HSA-5628897, TP53 Regulates Metabolic Genes R-HSA-611105, Respiratory electron transport R-HSA-9707564, Cytoprotection by HMOX1 |
SignaLinki | P14854 |
Miscellaneous databases
BioGRID-ORCSi | 1340, 220 hits in 1041 CRISPR screens |
ChiTaRSi | COX6B1, human |
GeneWikii | COX6B1 |
GenomeRNAii | 1340 |
Pharosi | P14854, Tbio |
PROi | PR:P14854 |
RNActi | P14854, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000126267, Expressed in apex of heart and 252 other tissues |
ExpressionAtlasi | P14854, baseline and differential |
Genevisiblei | P14854, HS |
Family and domain databases
CDDi | cd00926, Cyt_c_Oxidase_VIb, 1 hit |
Gene3Di | 1.10.10.140, 1 hit |
InterProi | View protein in InterPro IPR003213, Cyt_c_oxidase_su6B IPR036549, Cyt_c_oxidase_su6B_sf |
PANTHERi | PTHR11387, PTHR11387, 1 hit |
Pfami | View protein in Pfam PF02297, COX6B, 1 hit |
PIRSFi | PIRSF000278, Cyt_c_oxidase_6B, 1 hit |
SUPFAMi | SSF47694, SSF47694, 1 hit |
PROSITEi | View protein in PROSITE PS51808, CHCH, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | CX6B1_HUMAN | |
Accessioni | P14854Primary (citable) accession number: P14854 Secondary accession number(s): B2R5C9, Q6IBL4 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | April 1, 1990 |
Last sequence update: | January 23, 2007 | |
Last modified: | February 23, 2022 | |
This is version 192 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
3D-structure, Reference proteomeDocuments
- Human chromosome 19
Human chromosome 19: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families