Homo sapiens Protein: UQCRB
Summary
InnateDB Protein IDBP-29386.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol UQCRB
Protein Name ubiquinol-cytochrome c reductase binding protein
Synonyms MC3DN3; QCR7; QP-C; QPC; UQBC; UQBP; UQCR6; UQPC;
Species Homo sapiens
Ensembl Protein ENSP00000287022
InnateDB Gene IDBG-29384 (UQCRB)
Protein Structure
UniProt Annotation
Function 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 component is involved in redox-linked proton pumping.
Subcellular Localization Mitochondrion inner membrane.
Disease Associations Mitochondrial complex III deficiency, nuclear 3 (MC3DN3) [MIM:615158]: A 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. {ECO:0000269PubMed:12709789}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 26 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
Experimentally validated
Total 26 [view]
Protein-Protein 26 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 1 [view]
Gene Ontology

Molecular Function
Accession GO Term
Biological Process
GO:0006119 oxidative phosphorylation
GO:0006122 mitochondrial electron transport, ubiquinol to cytochrome c
GO:0009060 aerobic respiration
GO:0022904 respiratory electron transport chain
GO:0044237 cellular metabolic process
GO:0044281 small molecule metabolic process
GO:0055114 oxidation-reduction process
Cellular Component
GO:0005743 mitochondrial inner membrane
GO:0005746 mitochondrial respiratory chain
GO:0005750 mitochondrial respiratory chain complex III
Protein Structure and Domains
PDB ID
InterPro IPR003197 Cytochrome b-c1 complex subunit 7
PFAM PF02271
PRINTS
PIRSF PIRSF000022
SMART
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt P14927
PhosphoSite PhosphoSite-P14927
TrEMBL
UniProt Splice Variant
Entrez Gene 7381
UniGene Hs.663220
RefSeq NP_006285
HUGO HGNC:12582
OMIM 191330
CCDS CCDS6269
HPRD 11770
IMGT
EMBL AP003465 BC005230 BU535281 CH471060 CR542176 CR542196 M22348 M26700 M26701 M26706 M26707 M26730 M35761 M37387 X13585
GenPept AAA60235 AAA60236 AAA60237 AAA60238 AAA60361 AAH05230 CAA31926 CAG46973 CAG46993 EAW91749