PDSS1 Proteins

Protein Information

PDSS1 is an enzyme that elongates the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. PDSS1 catalyzes the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate in the assembly of polyisoprenoid side chains, the first step in coenzyme Q biosynthesis. The protein may be peripherally associated with the inner mitochondrial membrane, though no transit peptide has been definitively identified to date. Defects in PDSS1 gene are a cause of coenzyme Q10 deficiency.The protein encoded by this gene is an enzyme that elongates the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. The gene product catalyzes the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate in the assembly of polyisoprenoid side chains, the first step in coenzyme Q biosynthesis. The protein may be peripherally associated with the inner mitochondrial membrane, though no transit peptide has been definitively identified to date. Defects in this gene are a cause of coenzyme Q10 deficiency.

Synonyms

2610203G20Rik; 2700031G06Rik; all-trans-decaprenyl-diphosphate synthase subunit 1; coenzyme Q1 homolog; COQ1; COQ10D2; Decaprenyl pyrophosphate synthase subunit 1; decaprenyl pyrophosphate synthetase subunit 1; decaprenyl-diphosphate synthase subunit 1; DPS; DPS1; hDPS1; mDLP1; MGC70953; mSPS1; PDSS1; polyprenyl pyrophosphate synthetase; prenyl (decaprenyl) diphosphate synthase, subunit 1; prenyl (solanesyl) diphosphate synthase, subunit 1; RP13-16H11.3; solanesyl-diphosphate synthase subunit 1; SPS; Sps1; subunit 1 of decaprenyl diphosphate synthase; subunit 1 of solanesyl diphosphate synthase; Tprt; TPT; TPT 1; trans-prenyltransferase (TPT); trans-prenyltransferase 1