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.
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Anti-UQCRC1 antibodies

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Mouse Anti-UQCRC1 Recombinant Antibody (11A51H12) (CBMAB-C4726-CN)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Cattle, C. elegans, Drosophila, Human, Mouse, Rat, Zebrafish
Clone: 11A51H12
Application*: IP
Datasheet SDS

Mouse Anti-UQCRC1 Recombinant Antibody (2D10) (CBMAB-C4731-CN)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 2D10
Application*: FC, WB
Datasheet SDS

Mouse Anti-UQCRC1 Recombinant Antibody (1G6) (CBMAB-C4730-CN)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Mouse, Rat, Dog
Clone: 1G6
Application*: WB, FC, IH, P
Datasheet SDS

Mouse Anti-UQCRC1 Recombinant Antibody (1G2) (CBMAB-C4729-CN)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1G2
Application*: IF, WB
Datasheet SDS

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

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1A3
Application*: FC, WB
Datasheet SDS

Mouse Anti-UQCRC1 Recombinant Antibody (16D10AD9AH5) (CBMAB-C4727-CN)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Cattle, Human, Mouse, Rat
Clone: 16D10AD9AH5
Application*: FC, ICC, IF, E, WB
Datasheet SDS

Mouse Anti-UQCRC1 Recombinant Antibody (CBFYU-252) (CBMAB-U0252-FY)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Mouse, Rat, Cattle, Human
Clone: CBFYU-252
Application*: WB, F, IF
Datasheet SDS

Mouse Anti-UQCRC1 Monoclonal Antibody (1311) (CBMAB-1689-YC)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human, Mouse, Rat
Clone: 1311
Application*: IC, IF, WB
Datasheet SDS

Mouse Anti-UQCRC1 Monoclonal Antibody (139) (CBMAB-1688-YC)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Mouse, Rat
Clone: 139
Application*: IC, IF, WB
Datasheet SDS

Mouse Anti-UQCRC1 Recombinant Antibody (CBT2515) (V2LY-0625-LY1690)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBT2515
Application*: WB, IH, IC, F
Datasheet SDS

Mouse Anti-UQCRC1 Recombinant Antibody (CBT3939) (V2LY-0625-LY1689)

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Target: UQCRC1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBT3939
Application*: WB, IH, IC, F
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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