Mouse Anti-UQCRC1 Recombinant Antibody (1A3) (CBMAB-C4728-CN)

Ubiquinol-Cytochrome C Reductase Core Protein 1

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 protein may mediate formation of the complex between cytochromes c and c1.

Ubiquinol-Cytochrome C Reductase Core Protein 1; Ubiquinol-Cytochrome-C Reductase Complex Core Protein 1; Ubiquinol-Cytochrome C Reductase Core Protein I; Complex III Subunit 1; Core Protein I; Cytochrome B-C1 Complex Subunit 1, Mitochondrial; D3S3191; UQCR1; QCR1;

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 (By similarity).
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 (By similarity).
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). Seems to play an important role in the maintenance of proper mitochondrial function in nigral dopaminergic neurons (PubMed:33141179).

Biological Process aerobic respiration Source:ProtInc1 Publication
Biological Process cellular respiration Source:ComplexPortal1 Publication
Biological Process mitochondrial electron transport, ubiquinol to cytochrome c Source:ComplexPortal1 Publication
Biological Process oxidative phosphorylation Source:ProtInc1 Publication
Biological Process response to activity Source:Ensembl
Biological Process response to alkaloid Source:Ensembl

Mitochondrion inner membrane

Parkinsonism with polyneuropathy (PKNPY):
An autosomal dominant disorder characterized by late-onset, levodopa-responsive parkinsonism with asymmetric tremor, rigidity and bradykinesia. Patients also manifest a sensorimotor polyneuropathy with variable degrees of distal legs and hands muscle atrophy and weakness, and absent deep tendon reflexes.