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Prenyl (decaprenyl) diphosphate synthase, subunit 1
Symbols PDSS1 ; COQ1; COQ10D2; DPS; SPS; TPRT; TPT; TPT 1; hDPS1
External IDs OMIM607429 MGI1889278 HomoloGene5353 GeneCards: PDSS1 Gene
EC number
RNA expression pattern
PBB GE PDSS1 220865 s at tn.png
More reference expression data
Species Human Mouse
Entrez 23590 56075
Ensembl ENSG00000148459 ENSMUSG00000026784
UniProt Q5T2R2 Q33DR2
RefSeq (mRNA) NM_014317 NM_019501
RefSeq (protein) NP_055132 NP_062374
Location (UCSC) Chr 10:
26.7 – 26.75 Mb
Chr 2:
22.9 – 22.94 Mb
PubMed search [1] [2]

Decaprenyl-diphosphate synthase subunit 1 is an enzyme that in humans is encoded by the PDSS1 gene.[1][2]

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.[2]


  1. ^ Rotig A, Appelkvist EL, Geromel V, Chretien D, Kadhom N, Edery P, Lebideau M, Dallner G, Munnich A, Ernster L, Rustin P (Sep 2000). "Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency". Lancet 356 (9227): 391–395. doi:10.1016/S0140-6736(00)02531-9. PMID 10972372. 
  2. ^ a b "Entrez Gene: PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1". 

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