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UQCRC1

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.
Full Name
Ubiquinol-Cytochrome C Reductase Core Protein 1
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 (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
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
Cellular Location
Mitochondrion inner membrane
Involvement in disease
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.

Anti-UQCRC1 antibodies

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Mouse, Rat, Cattle, Human
Clone: CBFYU-252
Application*: WB, F, IF
Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Cattle, C. elegans, Drosophila, Human, Mouse, Rat, Zebrafish
Clone: 11A51H12
Application*: IP
Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Cattle, Human, Mouse, Rat
Clone: 16D10AD9AH5
Application*: FC, ICC, IF, E, WB
Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1A3
Application*: FC, WB
Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1G2
Application*: IF, WB
Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Mouse, Rat, Dog
Clone: 1G6
Application*: WB, FC, IH, P
Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 2D10
Application*: FC, WB
Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Mouse, Rat
Clone: 139
Application*: IC, IF, WB
Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human, Mouse, Rat
Clone: 1311
Application*: IC, IF, WB
More Infomation
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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