PDSS1
Function
Heterotetrameric enzyme that catalyzes the condensation of farnesyl diphosphate (FPP), which acts as a primer, and isopentenyl diphosphate (IPP) to produce prenyl diphosphates of varying chain lengths and participates in the determination of the side chain of ubiquinone (PubMed:16262699). Supplies nona and decaprenyl diphosphate, the precursors for the side chain of the isoprenoid quinones ubiquinone-9 (Q9)and ubiquinone-10 (Q10) respectively (PubMed:16262699). The enzyme adds isopentenyl diphosphate molecules sequentially to farnesyl diphosphate with trans stereochemistry (PubMed:16262699).
Involvement in disease
Coenzyme Q10 deficiency, primary, 2
COQ10D2
An autosomal recessive multisystem disorder characterized by early-onset deafness, optic atrophy, mild intellectual disability, peripheral neuropathy, obesity, livedo reticularis, and cardiac valvulopathy.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Cofactor biosynthesis; ubiquinone biosynthesis.
Sequence Similarities
Belongs to the FPP/GGPP synthase family.
Cellular localization
- Mitochondrion
Alternative names
DPS1, TPRT, PDSS1, All trans-polyprenyl-diphosphate synthase PDSS1, All-trans-decaprenyl-diphosphate synthase subunit 1, Decaprenyl pyrophosphate synthase subunit 1, Decaprenyl-diphosphate synthase subunit 1, Solanesyl-diphosphate synthase subunit 1, Trans-prenyltransferase 1, TPT 1