PDSS1 explained

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.

Further reading

• Appelkvist EL . Regulation of coenzyme Q biosynthesis . Mol. Aspects Med. . 15 Suppl . s37–46 . 1995 . 7752843 . 10.1016/0098-2997(94)90011-6. vanc. Aberg F . Guan Z . 3 . Parmryd . I . Dallner . G .
• Runquist M . Isoprenoid biosynthesis in rat liver mitochondria. Studies on farnesyl pyrophosphate synthase and trans-prenyltransferase . J. Biol. Chem. . 269 . 8 . 5804–9 . 1994 . 8119922 . vanc. Ericsson J . Thelin A . 3 . Chojnacki . T . Dallner . G . 10.1016/S0021-9258(17)37533-6 . free .
• Maruyama K, Sugano S . Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides . Gene . 138 . 1–2 . 171–174 . 1994 . 8125298 . 10.1016/0378-1119(94)90802-8 .
• Teclebrhan H, Olsson J, Swiezewska E, Dallner G . Biosynthesis of the side chain of ubiquinone:trans-prenyltransferase in rat liver microsomes . J. Biol. Chem. . 268 . 31 . 23081–6 . 1993 . 10.1016/S0021-9258(19)49428-3 . 8226825 . free .
• Suzuki Y . Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library . Gene . 200 . 1–2 . 149–156 . 1997 . 9373149 . 10.1016/S0378-1119(97)00411-3 . vanc. Yoshitomo-Nakagawa K . Maruyama K . 3 . Suyama . A . Sugano . S .
• Strausberg RL . Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences . Proc. Natl. Acad. Sci. U.S.A. . 99 . 26 . 16899–16903 . 2003 . 12477932 . 10.1073/pnas.242603899 . 139241 . vanc. Feingold EA . Grouse LH . 3 . Derge . JG . Klausner . RD . Collins . FS . Wagner . L . Shenmen . CM . Schuler . GD . 2002PNAS...9916899M . free .
• Deloukas P . The DNA sequence and comparative analysis of human chromosome 10 . Nature . 429 . 6990 . 375–381 . 2004 . 15164054 . 10.1038/nature02462 . vanc. Earthrowl ME . Grafham DV . 3 . Rubenfield . M. . French . L. . Steward . C. A. . Sims . S. K. . Jones . M. C. . Searle . S. . 2004Natur.429..375D . free .
• Gerhard DS . The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC) . Genome Res. . 14 . 10B . 2121–2127 . 2004 . 15489334 . 10.1101/gr.2596504 . 528928 . vanc. Wagner L . Feingold EA . 3 . Shenmen . CM . Grouse . LH . Schuler . G . Klein . SL . Old . S . Rasooly . R .
• Saiki R . Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans . FEBS J. . 272 . 21 . 5606–5622 . 2005 . 16262699 . 10.1111/j.1742-4658.2005.04956.x . vanc. Nagata A . Kainou T . 3 . Matsuda . Hideyuki . Kawamukai . Makoto . 13735213 . free .
• Mollet J . Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders . J. Clin. Invest. . 117 . 3 . 765–772 . 2007 . 17332895 . 10.1172/JCI29089 . 1804361 . vanc. Giurgea I . Schlemmer D . 3 . Dallner . Gustav . Chretien . Dominique . Delahodde . Agnès . Bacq . Delphine . De Lonlay . Pascale . Munnich . Arnold .

Notes and References

1. Rotig A, Appelkvist EL, Geromel V, Chretien D, Kadhom N, Edery P, Lebideau M, Dallner G, Munnich A, Ernster L, Rustin P . Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency . Lancet . 356 . 9227 . 391–395 . Sep 2000 . 10972372 . 10.1016/S0140-6736(00)02531-9 . 27320145 .
2. Web site: Entrez Gene: PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1.