Human COQ2 (4-hydroxybenzoate polyprenyltransferase, mitochondrial) ELISA KIT (HUFI08257)

The Human COQ2 ELISA kit from Assay Genie is a research-use tool for quantitative measurement of 4-hydroxybenzoate polyprenyltransferase, the inner-mitochondrial-membrane enzyme encoded by the COQ2 gene. COQ2 catalyzes the prenylation of 4-hydroxybenzoate in the coenzyme Q10 biosynthesis pathway, attaching the long polyisoprenoid tail that defines ubiquinone. Because CoQ10 abundance reflects mitochondrial bioenergetic capacity, this assay supports studies of oxidative phosphorylation, electron transport chain function, and mitochondrial disease models.

The kit is relevant for investigations of primary coenzyme Q10 deficiency, a clinically heterogeneous disorder linked to biallelic COQ2 variants and associated with steroid-resistant nephrotic syndrome, cerebellar ataxia, encephalomyopathy, and hypertrophic cardiomyopathy. COQ2 polymorphisms have also been studied in the context of multiple system atrophy, where reduced ubiquinone biosynthesis may contribute to neurodegeneration. Researchers measuring mitochondrial enzyme abundance in patient-derived lymphoblasts, skeletal muscle homogenates, or cellular respiration models can use this reagent alongside functional CoQ10 quantification.

Each kit is supplied with a precoated microplate, biotin-conjugated detection antibody, streptavidin-HRP reagents, and standards calibrated to recombinant human COQ2 protein. Store unopened kits at refrigerated conditions, protect from light, and avoid repeated freeze-thaw cycles of individual components to preserve antibody binding activity.