PDSS1

Prenyl (decaprenyl) diphosphate synthase, subunit 1
Identifiers
Symbols	PDSS1; COQ1; DPS; MGC70953; RP13-16H11.3; SPS; TPRT; TPT; TPT 1; hDPS1
External IDs	OMIM: 607429 MGI: 1889278 HomoloGene: 5353 GeneCards: PDSS1 Gene
EC number	2.5.1.91
Gene Ontology Molecular function • trans-hexaprenyltranstransferase activity • transferase activity • metal ion binding • protein heterodimerization activity • trans-octaprenyltranstransferase activity Cellular component • mitochondrion Biological process • ubiquinone biosynthetic process • isoprenoid biosynthetic process • protein heterotetramerization Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	23590	56075
Ensembl	ENSG00000148459	ENSMUSG00000026784
UniProt	Q5T2R2	Q9CZQ1
RefSeq (mRNA)	NM_014317	NM_019501.3
RefSeq (protein)	NP_055132	NP_062374.2
Location (UCSC)	Chr 10: 26.99 – 27.04 Mb	Chr 2: 22.75 – 22.8 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]

References

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". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=23590. 

Further reading

• Appelkvist EL, Aberg F, Guan Z et al. (1995). "Regulation of coenzyme Q biosynthesis". Mol. Aspects Med. 15 Suppl: s37–46. PMID 7752843. 
• 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. PMID 8119922. 
• Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene 138 (1–2): 171–174. doi:10.1016/0378-1119(94)90802-8. PMID 8125298. 
• 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. PMID 8226825. 
• 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". Gene 200 (1–2): 149–156. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149. 
• Strausberg RL, Feingold EA, Grouse LH et al. (2003). "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. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=139241. 
• Deloukas P, Earthrowl ME, Grafham DV et al. (2004). "The DNA sequence and comparative analysis of human chromosome 10". Nature 429 (6990): 375–381. doi:10.1038/nature02462. PMID 15164054. 
• Gerhard DS, Wagner L, Feingold EA et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–2127. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=528928. 
• Saiki R, Nagata A, Kainou T et al. (2005). "Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans". FEBS J. 272 (21): 5606–5622. doi:10.1111/j.1742-4658.2005.04956.x. PMID 16262699. 
• Mollet J, Giurgea I, Schlemmer D et al. (2007). "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. doi:10.1172/JCI29089. PMC 1804361. PMID 17332895. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1804361.