Recombinant Human Cytochrome b-c1 complex subunit 2, mitochondrial (UQCRC2)

Uniprot Id

P22695

Target Species

Human

Target Name

UQCRC2

Target Full Name

Cytochrome b-c1 complex subunit 2, mitochondrial

Target 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. The 2 core subunits UQCRC1/QCR1 and UQCRC2/QCR2 are homologous to the 2 mitochondrial-processing peptidase (MPP) subunits beta-MPP and alpha-MPP respectively, and they seem to have preserved their MPP processing properties. May be involved in the in situ processing of UQCRFS1 into the mature Rieske protein and its mitochondrial targeting sequence (MTS)/subunit 9 when incorporated into complex III (Probable).

Target Involvement

Mitochondrial complex III deficiency, nuclear 5 (MC3DN5)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Peptidase M16 family, UQCRC2/QCR2 subfamily

Target Synonyms

Complex III subunit 2; Core protein II; Cytochrome b c1 complex subunit 2 mitochondrial; Cytochrome b-c1 complex subunit 2; EC 1.10.2.2; MC3DN5; mitochondrial; QCR2; QCR2_HUMAN; Ubiquinol cytochrome c reductase complex core protein 2 mitochondrial; Ubiquinol cytochrome c reductase core protein II; Ubiquinol-cytochrome-c reductase complex core protein 2; UQCR2; Uqcrc2

Target Background

The protein encoded by this gene is located in the mitochondrion, where it is part of the ubiquinol-cytochrome c reductase complex (also known as complex III). This complex constitutes a part of the mitochondrial respiratory chain. Defects in this gene are a cause of mitochondrial complex III deficiency nuclear type 5.