PDSS1
Description
The PDSS1 (decaprenyl diphosphate synthase subunit 1) is a protein-coding gene located on chromosome 10.
PDSS1 is a gene that encodes an enzyme involved in the production of coenzyme Q, a crucial component of the respiratory chain. The protein encoded by PDSS1 elongates the prenyl side chain of coenzyme Q, catalyzing the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate. This process is the first step in coenzyme Q biosynthesis. While PDSS1 may be peripherally associated with the inner mitochondrial membrane, a definitive transit peptide has not been identified.
PDSS1, along with its partner subunit PDSS2, forms a heterotetrameric enzyme that plays a critical role in the biosynthesis of ubiquinone (coenzyme Q). This enzyme catalyzes the sequential addition of isopentenyl diphosphate (IPP) units to farnesyl diphosphate (FPP), the primer molecule, to generate prenyl diphosphates of varying chain lengths. The trans stereochemistry of this addition ensures the correct configuration of the final product. Specifically, PDSS1 contributes to the production of nona- and decaprenyl diphosphate, precursors for ubiquinone-9 (Q9) and ubiquinone-10 (Q10), respectively. These are essential components of the mitochondrial respiratory chain, where they function as electron carriers in oxidative phosphorylation.
PDSS1 is also known as COQ1, COQ10D2, COQ1A, DPS, SPS, TPRT, TPT, TPT 1, hDPS1.
