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Antibody Product Overview
| ELK.No | ES17299 |
| Product name | COQ2 rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB |
| Other name | |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 27235 |
| Human Swiss-Prot | Q96H96 |
| Source | Rabbit |
| Isotype | IgG |
| Target | COQ2 |
| Fields | >>Ubiquinone and other terpenoid-quinone biosynthesis;>>Metabolic pathways;>>Biosynthesis of cofactors |
| Gene name | COQ2 CL640 |
| Protein name | COQ2 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 71883 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q66JT7 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 498332 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q499N4 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human COQ2 AA range: 160-210 |
| Specificity | This antibody detects endogenous levels of COQ2 at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1：500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 41kD |
| Observed band (KD) | |
| Background | This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant. This enzyme, which is part of the coenzyme Q10 pathway, catalyzes the prenylation of parahydroxybenzoate with an all-trans polyprenyl group. Mutations in this gene cause coenzyme Q10 deficiency, a mitochondrial encephalomyopathy, and also COQ2 nephropathy, an inherited form of mitochondriopathy with primary renal involvement. [provided by RefSeq, Oct 2009], |
| Function | disease:Defects in COQ2 are a cause of coenzyme Q10 deficiency [MIM:607426]. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy.,function:Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB.,pathway:Cofactor biosynthesis; ubiquinone biosynthesis.,similarity:Belongs to the ubiA prenyltransferase family.,tissue specificity:Widely expressed. Present in all of the tissues tested. Expressed at higher level in skeletal muscle, adrenal glan |
| Subcellular location | Mitochondrion inner membrane ; Multi-pass membrane protein ; Matrix side . |
| Expression | Widely expressed. Present in all of the tissues tested. Expressed at higher level in skeletal muscle, adrenal glands and the heart. |
Additional Images
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Western blot analysis of lysates from SW480 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES17299-50
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