Product Description

COQ2 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES17299-50, ES17299-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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],

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from human COQ2 AA range: 160-210

Isotype: IgG

Target: COQ2

Specificity: This antibody detects endogenous levels of COQ2 at Human/Mouse/Rat

Dilution: WB 1：500-2000

Purification: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Expression: Widely expressed, Present in all of the tissues tested, Expressed at higher level in skeletal muscle, adrenal glands and the heart,

Molecular Weight (Da): 41kD

Fields: Ubiquinone and other terpenoid-quinone biosynthesis, Metabolic pathways, Biosynthesis of cofactors

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

Storage and Stability: -20°C/1 year

Gene Name: COQ2 CL640

Protein Name: COQ2

Mouse Gene ID: 71883

Mouse SwissProt Number: Q66JT7

Rat Gene ID: 498332

Rat SwissProt Number: Q499N4

Research Use Only