Decaprenyl diphosphate synthase subunit 1 (PDSS1)

Decaprenyl diphosphate synthase subunit 1 (PDSS1) is an enzyme that catalyzes the formation and elongation of the prenyl (decaprenyl) side chain of coenzyme Q10 (ubiquinone), a crucial component of the mitochondrial electron transport chain. PDSS1 acts early in the CoQ10 biosynthesis pathway, condensing isopentenyl diphosphate (IPP) molecules onto a farnesyl diphosphate primer to produce all-trans-decaprenyl diphosphate, which determines the final length of the CoQ10 side chain. PDSS1 is peripherally associated with the inner mitochondrial membrane and functions as part of a heterotetrameric complex with other subunits (e.g., PDSS2) to facilitate correct chain length. Mutations in PDSS1 cause primary CoQ10 deficiency, clinically manifesting in a range of mitochondrial disorders. In cancer biology, PDSS1 is implicated in oncogenic signaling, particularly in triple-negative breast cancer, where its upregulation associates with metastasis and poor prognosis. While CoQ10 supplementation is used therapeutically in deficiency states, no direct pharmacological inhibitors of PDSS1 are clinically available.

Other names

All trans-polyprenyl-diphosphate synthase PDSS1DPS1TPRTTPT 1TPTCOQ1COQ1AAll-trans-decaprenyl-diphosphate synthase subunit 1Decaprenyl pyrophosphate synthase subunit 1Decaprenyl-diphosphate synthase subunit 1Solanesyl-diphosphate synthase subunit 1Trans-prenyltransferase 1coenzyme Q1 homolog (yeast)COQ10D2DPSSPShDPS1all trans-polyprenyl-diphosphate synthase PDSS1all-trans-decaprenyl-diphosphate synthase subunit 1coenzyme Q1 homologdecaprenyl pyrophosphate synthase subunit 1polyprenyl pyrophosphate synthetaseprenyl (decaprenyl) diphosphate synthase, subunit 1solanesyl-diphosphate synthase subunit 1subunit 1 of decaprenyl diphosphate synthasetrans-prenyltransferase (TPT)trans-prenyltransferase 1PDSS1

Coenzyme Q10 supplementation aims to restore deficient electron transport and antioxidant activity due to PDSS1 dysfunction.