Human PDSS2 ELISA Kit (HUFI07278)

Quantify endogenous levels of prenyl diphosphate synthase subunit 2 (PDSS2), the mitochondrial matrix enzyme that catalyzes decaprenyl diphosphate synthesis in the coenzyme Q10 biosynthesis pathway, with this in vitro research ELISA from Assay Genie. PDSS2 forms a heterotetrameric complex with PDSS1 and supplies the polyisoprenoid tail required for ubiquinone assembly, making it relevant to studies of primary coenzyme Q10 deficiency, mitochondrial respiration, and oxidative phosphorylation disorders. Biallelic PDSS2 mutations have been identified in patients presenting with steroid-resistant nephrotic syndrome, Leigh syndrome, and early-onset encephalopathy, and the protein is used as a biomarker for CoQ pathway dysfunction in these conditions. The kit is supplied in a microplate format supporting 96 individual determinations, intended for research use in academic, translational, and clinical laboratory settings.