PDSS1

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; OMA:PDSS1 - orthologs
Gene location (Human)
Chr.	Chromosome 10 (human)
End	26,746,798 bp
Gene location (Mouse)
Chr.	Chromosome 2 (mouse)
End	22,830,278 bp
RNA expression pattern
	Top expressed in
	rectum; ; jejunal mucosa; ; duodenum; ; monocyte; ; cerebellar hemisphere; ; ganglionic eminence; ; gastrocnemius muscle; ; Achilles tendon; ; upper lobe of left lung; ; right lobe of liver;
	Top expressed in
	Paneth cell; ; proximal tubule; ; otic placode; ; saccule; ; soleus muscle; ; duodenum; ; jejunum; ; yolk sac; ; myocardium of ventricle; ; atrioventricular valve;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	metal ion binding; protein heterodimerization 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
	23590
	56075
	ENSG00000148459
	ENSMUSG00000026784
	Q5T2R2
	Q33DR2
	NM_014317; NM_001321978; NM_001321979
	NM_019501; NM_001355467
	NP_001308907; NP_001308908; NP_055132
	NP_062374; NP_001342396
	Wikidata
View/Edit Human	View/Edit Mouse

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

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

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000148459 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000026784 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ 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. S2CID 27320145.
^ ^a ^b "Entrez Gene: PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1".

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. doi:10.1016/S0021-9258(17)37533-6. 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. doi:10.1016/S0021-9258(19)49428-3. 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. Bibcode:2002PNAS...9916899M. 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. Bibcode:2004Natur.429..375D. 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. S2CID 13735213.
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.

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[refGRCh38Ensembl-1] GRCh38: Ensembl release 89: ENSG00000148459 – Ensembl, May 2017

[refGRCm38Ensembl-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.

[pmid10972372-5] 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. S2CID 27320145.

[entrez-6] "Entrez Gene: PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1".

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References

Further reading