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MGP003276

UniProt Annotations

Entry Information
Gene Nameubiquinol-cytochrome c reductase core protein II
Protein EntryQCR2_HUMAN
UniProt IDP22695
SpeciesHuman
Comments
Comment typeDescription
CautionDoes not seem to have a protease activity as it lack the zinc-binding site. {ECO:0000305}.
DiseaseMitochondrial complex III deficiency, nuclear 5 (MC3DN5) [MIM:615160]: 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:23281071}. 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. The core protein 2 is required for the assembly of the complex.
InteractionO00555:CACNA1A; NbExp=2; IntAct=EBI-1051424, EBI-766279;
SimilarityBelongs to the peptidase M16 family. UQCRC2/QCR2 subfamily. {ECO:0000305}.
Subcellular LocationMitochondrion inner membrane; Peripheral membrane protein; Matrix side.
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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