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],

• Gene ID:

  27235

• UniProt:

  Q96H96

• Cellular Locus:

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

• Host:

  Rabbit

• Species Reactivity:

  Human, Mouse, Rat

• Reactivity:

  Human; Mouse; Rat

• Immunogen:

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

• Clonality:

  Polyclonal

• Isotype:

  IgG

• Source:

  Rabbit

• Applications:

  WB

• Validated Applications:

  WB

• Stability:

  -20°C/1 year

• Concentration:

  1 mg/mL

• Dilution:

  WB 1: 500-2000

• Storage Conditions:

  PBS with 0.02% sodium azide and 50% glycerol pH 7.4. Store at -20°C. Avoid repeated freeze-thaw cycles.

• Subcellular Location:

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

• Gene ID (Human):

  27235

• SwissProt (Human):

  Q96H96