Immunogen
Synthesized peptide derived from human QCR8 AA range: 2-52
Specificity
This antibody detects endogenous levels of QCR8 at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
IHC-p 1：50-200. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
This gene encodes a ubiquinone-binding protein of low molecular mass. This protein is a small core-associated protein and a subunit of ubiquinol-cytochrome c reductase complex III, which is part of the mitochondrial respiratory chain. [provided by RefSeq, Jul 2008],
Function
disease:Defects in UQCRQ are a cause of mitochondrial complex III deficiency (CIII deficiency) [MIM:124000]. Affected individuals display severe psychomotor retardation and extrapyramidal signs. Neurologic features include dystonia, athetoid movements, ataxia, mild axial hypotonia, increased tone, hyperreflexia, and inability to walk unsupported. Serum lactate is mildly elevated and muscle biopsies show a reduction in mitochondrial complex III activity.,function: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 subunit, together with cytochrome b, binds to ubiquinone.,similarity:Belongs to the UQCRQ/QCR8 family.,subunit:The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QC
Fields
>>Oxidative phosphorylation;>>Metabolic pathways;>>Cardiac muscle contraction;>>Thermogenesis;>>Non-alcoholic fatty liver disease;>>Alzheimer disease;>>Parkinson disease;>>Amyotrophic lateral sclerosis;>>Huntington disease;>>Prion disease;>>Pathways of neurodegeneration - multiple diseases;>>Chemical carcinogenesis - reactive oxygen species;>>Diabetic cardiomyopathy
Human protein sequence Database
O14949
Mouse protein sequence database
Q9CQ69
Rat protein sequence database
Q7TQ16
Cellular localization
Mitochondrion inner membrane ; Single-pass membrane protein .
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