Decaprenyl diphosphate synthase subunit 2 (PDSS2)

Decaprenyl diphosphate synthase subunit 2 (PDSS2) is a mitochondrial enzyme that catalyzes the initial step of coenzyme Q10 biosynthesis by assembling polyisoprenoid side chains from isopentyl diphosphate, thus playing a critical role in cellular respiration and antioxidant defense[1][6]. Defects in PDSS2 are causative for coenzyme Q10 deficiency, impairing ATP generation and increasing oxidative stress, which manifest clinically as cardiovascular, neuromuscular, and neurodegenerative disorders, as well as some cancers where PDSS2 may act as a tumor suppressor[2][4][6][8]. Therapeutic approaches generally involve coenzyme Q10 supplementation rather than direct PDSS2 modulation, with diagnostic use focused on measuring PDSS2 expression or CoQ10 levels to monitor disease or therapy[2][6].

Other names

All trans-polyprenyl-diphosphate synthase PDSS2C6orf210DLP1bA59I9.3COQ1BAll-trans-decaprenyl-diphosphate synthase subunit 2Candidate tumor suppressor proteinDecaprenyl pyrophosphate synthase subunit 2Solanesyl-diphosphate synthase subunit 2COQ10D3hDLP1

Supplements aim to restore CoQ10 levels lost due to PDSS2 deficiency, thus restoring mitochondrial electron transport and cellular energy production Potential indirect modulation of PDSS2 activity by regulating upstream biosynthetic steps