Protein Name decaprenyl diphosphate synthase subunit 1
Organism Homo sapiens
Gene ID 23590
Gene Symbol

PDSS1

UniProt Q5T2R2 (DPS1_HUMAN)
Relationships Total Number of functionally related compound(s) : 74
Total Number of Articles : 47
Description

decaprenyl diphosphate synthase subunit 1

Gene Summary

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. [provided by RefSeq, Jul 2008]

synonyms
  • all trans-polyprenyl-diphosphate synthase PDSS1
  • all-trans-decaprenyl-diphosphate synthase subunit 1
  • coenzyme Q1 homolog
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Properties