COQ2 rabbit pAb

• 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]

• Description :

  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],

• UniProt :

  Q96H96

• Swiss Prot :

  Q96H96

• Reactivity :

  Human; Mouse; Rat

• Immunogen :

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

• Target :

  COQ2

• Clonality :

  Polyclonal

• Source :

  Rabbit

• Applications :

  WB

• Concentration :

  1 mg/ml

• Dilution :

  WB 1:500-2000

• Buffer :

  -20°C/1 year

• Storage Conditions :

  -20°C/1 year

• Fragment :

  IgG

• Subcellular Location :

  Mitochondrion inner membrane ; Multi-pass membrane protein ; Matrix side .

• Gene ID (Human) :

  27235