PDSS1

Decaprenyl-diphosphate synthase subunit 1 is an enzyme that in humans is encoded by the PDSS1 gene.[5][6]

PDSS1
Identifiers
Aliases	PDSS1, COQ1, COQ10D2, DPS, SPS, TPRT, TPT, TPT 1, hDPS1, prenyl (decaprenyl) diphosphate synthase, subunit 1, decaprenyl diphosphate synthase subunit 1, COQ1A
External IDs	OMIM: 607429 MGI: 1889278 HomoloGene: 5353 GeneCards: PDSS1
Gene location (Human) Chr. Chromosome 10 (human)[1] Band 10p12.1 Start 26,697,701 bp[1] End 26,746,798 bp[1]
Gene location (Mouse) Chr. Chromosome 2 (mouse)[2] Band 2|2 A3 Start 22,895,522 bp[2] End 22,940,266 bp[2]
RNA expression pattern More reference expression data
Gene ontology Molecular function • metal ion binding • protein heterodimerization activity • trans-octaprenyltranstransferase activity • trans-hexaprenyltranstransferase activity • transferase activity Cellular component • mitochondrial matrix • mitochondrion • transferase complex Biological process • isoprenoid biosynthetic process • protein heterotetramerization • ubiquinone biosynthetic process Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	23590	56075
Ensembl	ENSG00000148459	ENSMUSG00000026784
UniProt	Q5T2R2	Q33DR2
RefSeq (mRNA)	NM_014317 NM_001321978 NM_001321979	NM_019501 NM_001355467
RefSeq (protein)	NP_001308907 NP_001308908 NP_055132	NP_062374 NP_001342396
Location (UCSC)	Chr 10: 26.7 – 26.75 Mb	Chr 2: 22.9 – 22.94 Mb
PubMed search	[3]	[4]
Wikidata
View/Edit Human View/Edit Mouse

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.[6]

References

1. GRCh38: Ensembl release 89: ENSG00000148459 - Ensembl, May 2017
2. GRCm38: Ensembl release 89: ENSMUSG00000026784 - Ensembl, May 2017
3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
5. 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.
6. "Entrez Gene: PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1".

Further reading

• Appelkvist EL, Aberg F, Guan Z, et al. (1995). "Regulation of coenzyme Q biosynthesis". Mol. Aspects Med. 15 Suppl: s37–46. doi:10.1016/0098-2997(94)90011-6. 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.
• 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.
• 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.