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Mouse Anti-UQCRFS1 Recombinant Antibody (5A5) (CBMAB-C1044-CN)

This product is a Mouse antibody that recognizes UQCRFS1. The antibody 5A5 can be used for immunoassay techniques such as: WB, IHC-P, ICC, FC, IF.
See all UQCRFS1 antibodies

Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Cattle, Human
Clone
5A5
Antibody Isotype
IgG2b
Application
WB, IHC-P, ICC, FC, IF

Basic Information

Immunogen
Other Immunogen Type corresponding to Cow UQCRFS1/ RISP.
Specificity
Mouse, Rat, Cattle, Human
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Concentration
1 mg/mL

Target

Full Name
Ubiquinol-Cytochrome C Reductase, Rieske Iron-Sulfur Polypeptide 1
Introduction
Cytochrome b-c1 complex subunit Rieske, mitochondrial: Component of the mitochondrial ubiquinol-cytochrome c reductase complex dimer (complex III dimer), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis (PubMed:28673544). Incorporation of UQCRFS1 is the penultimate step in complex III assembly (By similarity).
Entrez Gene ID
Human7386
Mouse66694
Rat291103
Cattle287020
UniProt ID
HumanP47985
MouseQ9CR68
RatP20788
CattleP13272
Alternative Names
Ubiquinol-Cytochrome C Reductase, Rieske Iron-Sulfur Polypeptide 1; Cytochrome B-C1 Complex Subunit 5; Ubiquinol-Cytochrome C Reductase Iron-Sulfur Subunit; Rieske Iron-Sulfur Protein; Rieske Protein UQCRFS1; Complex III Subunit 5; EC 1.10.2.2;
Function
Cytochrome b-c1 complex subunit Rieske, mitochondrial
Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation (PubMed:31883641).
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 Rieske protein is a catalytic core subunit containing a [2Fe-2S] iron-sulfur cluster. It cycles between 2 conformational states during catalysis to transfer electrons from the quinol bound in the Q0 site in cytochrome b to cytochrome c1 (By similarity).
Incorporation of UQCRFS1 is the penultimate step in complex III assembly (PubMed:28673544).
Cytochrome b-c1 complex subunit 9
Component of the ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII). UQCRFS1 undergoes proteolytic processing once it is incorporated in the complex III dimer. One of the fragments, called subunit 9, corresponds to its mitochondrial targeting sequence (MTS). The proteolytic processing is necessary for the correct insertion of UQCRFS1 in the complex III dimer, but the persistence of UQCRFS1-derived fragments may prevent newly imported UQCRFS1 to be processed and assembled into complex III and is detrimental for the complex III structure and function.
Biological Process
Biological Process cellular respiration Source:ComplexPortal
Biological Process mitochondrial electron transport, ubiquinol to cytochrome c Source:GO_Central1 Publication
Biological Process mitochondrial respiratory chain complex III assembly Source:UniProtKB1 Publication
Biological Process respiratory electron transport chain Source:UniProtKB1 Publication
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex III deficiency, nuclear 10 (MC3DN10):
A form of mitochondrial complex III deficiency, a disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. MC3DN10 is an autosomal recessive form characterized by fetal bradycardia, poor feeding, hypotonia, hypertrophic cardiomyopathy, alopecia totalis, low mitochondrial complex III activity and lactic acidosis.
Topology
Mitochondrial matrix: 79-103
Helical: 104-140
Mitochondrial intermembrane: 141-274
PTM
Proteolytic processing is necessary for the correct insertion of UQCRFS1 in the complex III dimer. Several fragments are generated during UQCRFS1 insertion, most probably due to the endogenous matrix-processing peptidase (MPP) activity of the 2 core protein subunits UQCRC1/QCR1 and UQCRC2/QCR2, which are homologous to the 2 mitochondrial-processing peptidase (MPP) subunits beta-MPP and alpha-MPP respectively. The action of the protease is also necessary for the clearance of the UQCRFS1 fragments.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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