PDSS2
Description
The PDSS2 (decaprenyl diphosphate synthase subunit 2) is a protein-coding gene located on chromosome 6.
PDSS2, encoded by the PDSS2 gene, is an enzyme crucial for synthesizing the prenyl side-chain of coenzyme Q, also known as ubiquinone. Ubiquinone is a vital component of the respiratory chain. PDSS2 catalyzes the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate, initiating the assembly of polyisoprenoid side chains, the first step in coenzyme Q biosynthesis. Deficiencies in PDSS2 may be linked to Coenzyme Q10 deficiency.
PDSS2 is a heterotetrameric enzyme that combines farnesyl diphosphate (FPP) with isopentenyl diphosphate (IPP) to create prenyl diphosphates of varying chain lengths, influencing the ubiquinone side chain. It produces nona and decaprenyl diphosphate, precursors of ubiquinone-9 (Q9) and ubiquinone-10 (Q10), respectively. PDSS2 adds isopentenyl diphosphate molecules sequentially to farnesyl diphosphate with a trans stereochemical configuration. It may participate in cerebellar development and regulate mitochondrial respiratory chain function.
PDSS2 is also known as C6orf210, COQ10D3, COQ1B, DLP1, bA59I9.3, hDLP1.
