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Protein

Cytochrome b-c1 complex subunit 8

Gene

UQCRQ

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

Functioni

This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This subunit, together with cytochrome b, binds to ubiquinone.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Biological processElectron transport, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport

Names & Taxonomyi

Protein namesi
Recommended name:
Cytochrome b-c1 complex subunit 8
Alternative name(s):
Complex III subunit 8
Complex III subunit VIII
Ubiquinol-cytochrome c reductase complex 9.5 kDa protein
Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C
Gene namesi
Name:UQCRQ
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

EuPathDBiHostDB:ENSG00000164405.10
HGNCiHGNC:29594 UQCRQ
MIMi612080 gene
neXtProtiNX_O14949

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

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial complex III deficiency, nuclear 4 (MC3DN4)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance.
See also OMIM:615159
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04591145S → F in MC3DN4. 1 PublicationCorresponds to variant dbSNP:rs11544803EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNETi27089
MalaCardsiUQCRQ
MIMi615159 phenotype
OpenTargetsiENSG00000164405
Orphaneti1460 Isolated complex III deficiency
PharmGKBiPA142670637

Chemistry databases

DrugBankiDB08453 2-NONYL-4-HYDROXYQUINOLINE N-OXIDE
DB07778 FAMOXADONE
DB07401 METHYL (2Z)-2-(2-{[6-(2-CYANOPHENOXY)PYRIMIDIN-4-YL]OXY}PHENYL)-3-METHOXYACRYLATE
DB08330 METHYL (2Z)-3-METHOXY-2-{2-[(E)-2-PHENYLVINYL]PHENYL}ACRYLATE
DB08690 UBIQUINONE-2

Polymorphism and mutation databases

BioMutaiUQCRQ

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemoved1 Publication
ChainiPRO_00001935442 – 82Cytochrome b-c1 complex subunit 8Add BLAST81

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei16PhosphoserineCombined sources1
Modified residuei33N6-acetyllysine; alternateBy similarity1
Modified residuei33N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiO14949
MaxQBiO14949
PaxDbiO14949
PeptideAtlasiO14949
PRIDEiO14949
ProteomicsDBi48332
TopDownProteomicsiO14949

PTM databases

iPTMnetiO14949
PhosphoSitePlusiO14949
SwissPalmiO14949

Expressioni

Gene expression databases

BgeeiENSG00000164405 Expressed in 241 organ(s), highest expression level in cardiac ventricle
CleanExiHS_UQCRQ
GenevisibleiO14949 HS

Organism-specific databases

HPAiHPA046693
HPA053323

Interactioni

Subunit structurei

The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).

Protein-protein interaction databases

BioGridi117991, 85 interactors
ComplexPortaliCPX-560 Mitochondrial respiratory chain complex III
IntActiO14949, 17 interactors
STRINGi9606.ENSP00000367934

Structurei

Secondary structure

182
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliO14949
SMRiO14949
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the UQCRQ/QCR8 family.Curated

Phylogenomic databases

eggNOGiKOG4116 Eukaryota
ENOG41123BH LUCA
GeneTreeiENSGT00390000004029
HOGENOMiHOG000205681
HOVERGENiHBG001468
InParanoidiO14949
KOiK00418
OMAiILNGAHF
OrthoDBiEOG091G13Z0
PhylomeDBiO14949
TreeFamiTF300281

Family and domain databases

Gene3Di1.20.5.210, 1 hit
InterProiView protein in InterPro
IPR004205 Cyt_bc1_su8
IPR036642 Cyt_bc1_su8_sf
PANTHERiPTHR12119 PTHR12119, 1 hit
PfamiView protein in Pfam
PF02939 UcrQ, 1 hit
ProDomiView protein in ProDom or Entries sharing at least one domain
PD331499 UcrQ, 1 hit
SUPFAMiSSF81508 SSF81508, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O14949-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MGREFGNLTR MRHVISYSLS PFEQRAYPHV FTKGIPNVLR RIRESFFRVV
60 70 80
PQFVVFYLIY TWGTEEFERS KRKNPAAYEN DK
Length:82
Mass (Da):9,906
Last modified:January 23, 2007 - v4
Checksum:i5ED13ABDD2990DD2
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti73 – 82KNPAAYENDK → RIQLPMKMTNEQRIRMTVPC L in BAA23321 (Ref. 1) Curated10

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04591145S → F in MC3DN4. 1 PublicationCorresponds to variant dbSNP:rs11544803EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D50369 mRNA Translation: BAA23321.1
BC001390 mRNA Translation: AAH01390.1
BC090048 mRNA Translation: AAH90048.1
CCDSiCCDS34237.1
RefSeqiNP_055217.2, NM_014402.4
UniGeneiHs.146602

Genome annotation databases

EnsembliENST00000378665; ENSP00000367934; ENSG00000164405
ENST00000378667; ENSP00000367936; ENSG00000164405
ENST00000378670; ENSP00000367939; ENSG00000164405
GeneIDi27089
KEGGihsa:27089
UCSCiuc003kya.2 human

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D50369 mRNA Translation: BAA23321.1
BC001390 mRNA Translation: AAH01390.1
BC090048 mRNA Translation: AAH90048.1
CCDSiCCDS34237.1
RefSeqiNP_055217.2, NM_014402.4
UniGeneiHs.146602

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTEelectron microscopy3.40A/N2-82[»]
5XTHelectron microscopy3.90AA/AN2-82[»]
5XTIelectron microscopy17.40AA/AN2-82[»]
ProteinModelPortaliO14949
SMRiO14949
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi117991, 85 interactors
ComplexPortaliCPX-560 Mitochondrial respiratory chain complex III
IntActiO14949, 17 interactors
STRINGi9606.ENSP00000367934

Chemistry databases

DrugBankiDB08453 2-NONYL-4-HYDROXYQUINOLINE N-OXIDE
DB07778 FAMOXADONE
DB07401 METHYL (2Z)-2-(2-{[6-(2-CYANOPHENOXY)PYRIMIDIN-4-YL]OXY}PHENYL)-3-METHOXYACRYLATE
DB08330 METHYL (2Z)-3-METHOXY-2-{2-[(E)-2-PHENYLVINYL]PHENYL}ACRYLATE
DB08690 UBIQUINONE-2

PTM databases

iPTMnetiO14949
PhosphoSitePlusiO14949
SwissPalmiO14949

Polymorphism and mutation databases

BioMutaiUQCRQ

Proteomic databases

EPDiO14949
MaxQBiO14949
PaxDbiO14949
PeptideAtlasiO14949
PRIDEiO14949
ProteomicsDBi48332
TopDownProteomicsiO14949

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000378665; ENSP00000367934; ENSG00000164405
ENST00000378667; ENSP00000367936; ENSG00000164405
ENST00000378670; ENSP00000367939; ENSG00000164405
GeneIDi27089
KEGGihsa:27089
UCSCiuc003kya.2 human

Organism-specific databases

CTDi27089
DisGeNETi27089
EuPathDBiHostDB:ENSG00000164405.10
GeneCardsiUQCRQ
HGNCiHGNC:29594 UQCRQ
HPAiHPA046693
HPA053323
MalaCardsiUQCRQ
MIMi612080 gene
615159 phenotype
neXtProtiNX_O14949
OpenTargetsiENSG00000164405
Orphaneti1460 Isolated complex III deficiency
PharmGKBiPA142670637
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4116 Eukaryota
ENOG41123BH LUCA
GeneTreeiENSGT00390000004029
HOGENOMiHOG000205681
HOVERGENiHBG001468
InParanoidiO14949
KOiK00418
OMAiILNGAHF
OrthoDBiEOG091G13Z0
PhylomeDBiO14949
TreeFamiTF300281

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport

Miscellaneous databases

ChiTaRSiUQCRQ human
GenomeRNAii27089
PROiPR:O14949
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000164405 Expressed in 241 organ(s), highest expression level in cardiac ventricle
CleanExiHS_UQCRQ
GenevisibleiO14949 HS

Family and domain databases

Gene3Di1.20.5.210, 1 hit
InterProiView protein in InterPro
IPR004205 Cyt_bc1_su8
IPR036642 Cyt_bc1_su8_sf
PANTHERiPTHR12119 PTHR12119, 1 hit
PfamiView protein in Pfam
PF02939 UcrQ, 1 hit
ProDomiView protein in ProDom or Entries sharing at least one domain
PD331499 UcrQ, 1 hit
SUPFAMiSSF81508 SSF81508, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiQCR8_HUMAN
AccessioniPrimary (citable) accession number: O14949
Secondary accession number(s): Q5FVE2, Q9BV88, Q9T2V7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: January 23, 2007
Last modified: November 7, 2018
This is version 161 of the entry and version 4 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

3D-structure, Complete proteome, Direct protein sequencing, 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
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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