Products

COQ2 rabbit pAb
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ES17299
Size: Price:
50 µL $148.00
100 µL $248.00

Overview

Product name: COQ2 rabbit pAb
Reactivity: Human; Mouse;Rat
Source: Rabbit
Dilutions: WB 1:500-2000
Immunogen: Synthesized peptide derived from human COQ2 AA range: 160-210
Storage: -20°C/1 year
Clonality: Polyclonal
Isotype: IgG
Concentration: 1 mg/ml
Molecular Weight: 41kD
GeneID: 27235
Human Swiss-Prot No: Q96H96
Cellular localization: Mitochondrion inner membrane ; Multi-pass membrane protein ; Matrix side .
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],