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MGP003274

UniProt Annotations

Entry Information
Gene Nameubiquinol-cytochrome c reductase binding protein
Protein EntryQCR7_HUMAN
UniProt IDP14927
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P14927-1; Sequence=Displayed; Name=2; IsoId=P14927-2; Sequence=VSP_045601; Note=No experimental confirmation available.;
CautionWas originally thought to be the ubiquinone-binding protein (QP-C). {ECO:0000305}.
DiseaseMitochondrial 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:0000269|PubMed:12709789}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionThis 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.
SimilarityBelongs to the UQCRB/QCR7 family. {ECO:0000305}.
Subcellular LocationMitochondrion inner membrane.
SubunitThe 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).
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