Decaprenyl-diphosphate synthase subunit 1 is an enzyme that in humans is encoded by the PDSS1gene.
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.
^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". Lancet356 (9227): 391–395. doi:10.1016/S0140-6736(00)02531-9. PMID10972372.
Appelkvist EL, Aberg F, Guan Z et al. (1995). "Regulation of coenzyme Q biosynthesis". Mol. Aspects Med. 15 Suppl: s37–46. PMID7752843.
Runquist M, Ericsson J, Thelin A et al. (1994). "Isoprenoid biosynthesis in rat liver mitochondria. Studies on farnesyl pyrophosphate synthase and trans-prenyltransferase". J. Biol. Chem.269 (8): 5804–9. PMID8119922.
Teclebrhan H, Olsson J, Swiezewska E, Dallner G (1993). "Biosynthesis of the side chain of ubiquinone:trans-prenyltransferase in rat liver microsomes". J. Biol. Chem.268 (31): 23081–6. PMID8226825.
Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene200 (1–2): 149–156. doi:10.1016/S0378-1119(97)00411-3. PMID9373149.