UQCRQ

This gene encodes a ubiquinone-binding protein of low molecular mass. This protein is a small core-associated protein and a subunit of ubiquinol-cytochrome c reductase complex III, which is part of the mitochondrial respiratory chain. [provided by RefSeq, Jul 2008]

Full Name

Ubiquinol-Cytochrome C Reductase Complex III Subunit VII

Function

Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of 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. The cytochrome b-c1 complex catalyzes electron transfer from ubiquinol to cytochrome c, linking this redox reaction to translocation of protons across the mitochondrial inner membrane, with protons being carried across the membrane as hydrogens on the quinol. In the process called Q cycle, 2 protons are consumed from the matrix, 4 protons are released into the intermembrane space and 2 electrons are passed to cytochrome c.

Biological Process

Biological Process cellular respiration Source:ComplexPortal1 Publication
Biological Process cerebellar Purkinje cell layer development Source:Ensembl
Biological Process hippocampus development Source:Ensembl
Biological Process hypothalamus development Source:Ensembl
Biological Process midbrain development Source:Ensembl
Biological Process mitochondrial electron transport, ubiquinol to cytochrome c Source:GO_Central1 Publication
Biological Process pons development Source:Ensembl
Biological Process pyramidal neuron development Source:Ensembl
Biological Process subthalamus development Source:Ensembl
Biological Process thalamus development Source:Ensembl

Cellular Location

Mitochondrion inner membrane

Involvement in disease

Mitochondrial complex III deficiency, nuclear 4 (MC3DN4):
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.

Topology

Mitochondrial matrix: 2-39
Helical: 40-68
Mitochondrial intermembrane: 69-82