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MetaCyc Enzyme: decaprenyl diphosphate synthase

Synonyms: COQ1

Species: Homo sapiens

Subunit composition of decaprenyl diphosphate synthase = [PDSS1]2[PDSS2]2
         Decaprenyl-diphosphate synthase subunit 1 = PDSS1
         Decaprenyl-diphosphate synthase subunit 2 = PDSS2

Summary:
The main quinone in humans is ubiquinone-10, also known as coenzyme Q10, which contains a side chain of 10 prenyl units. The side chain is produced by the action of the enzyme decaprenyl diphosphate synthase. Coenzyme Q10 distributes electrons among the various dehydrogenases and cytochrome components of the respiratory chain and plays a vital role in oxidative phosphorylation [Mollet07].

The human decaprenyl diphosphate synthase is a heterotetramer composed of two copies each of two different subunits, encoded by PDSS1 and PDSS2. The genes have been identified based on their similarity to the genes from Schizosaccharomyces pombe and were cloned and sequenced [Saiki05].

When both genes were expressed together in an Escherichia coli strain a functional enzyme was produced that catalyzed the synthesis of ubiquinone-10, instead of ubiquinone-8 which is native to Escherichia coli.

When the genes encoding the subunits of the human enzyme were mixed with genes encoding subunits of the murine enzyme (which produces ubiquinone-9), namely, either mPDSS2 and hPDSS1 or hPDSS2 and mPDSS1 were co-expressed, both


EC Number: 2.5.1.91
(2E,6E)-farnesyl diphosphate + 7 isopentenyl diphosphate <=> all-trans-decaprenyl diphosphate + 7 diphosphate
The reaction direction shown, that is, A + B ↔ C + D versus C + D ↔ A + B, is in accordance with the Enzyme Commission system.
The reaction is physiologically favored in the direction shown.
In Pathways: all-trans-decaprenyl diphosphate biosynthesis , ubiquinol-10 biosynthesis (eukaryotic)
Subunit of decaprenyl diphosphate synthase: Decaprenyl-diphosphate synthase subunit 1
Synonyms: decaprenyl diphosphate synthase subunit 1, 2.5.1.-, Decaprenyl pyrophosphate synthetase subunit 1, TPT, Trans-prenyltransferase

Gene: PDSS1 Accession Number: HS07530 (MetaCyc)

Molecular Weight: 46.261 kD (from nucleotide sequence)
GO Terms:
Biological Process: GO:0008299 - isoprenoid biosynthetic process
Molecular Function: GO:0016740 - transferase activity


Unification Links: Entrez:AAD28559 , PhosphoSite:Q5T2R2 , Pride:Q5T2R2 , Protein Model Portal:Q5T2R2 , SMR:Q5T2R2 , String:9606.ENSP00000365388 , UniProt:Q5T2R2
Relationship Links: InterPro:IN-FAMILY:IPR000092 , InterPro:IN-FAMILY:IPR008949 , InterPro:IN-FAMILY:IPR017446 , Panther:IN-FAMILY:PTHR12001 , Pfam:IN-FAMILY:PF00348 , Prosite:IN-FAMILY:PS00444 , Prosite:IN-FAMILY:PS00723
Citations: [Rotig00, Appelkvist94] Subunit of decaprenyl diphosphate synthase: Decaprenyl-diphosphate synthase subunit 2
Synonyms: decaprenyl diphosphate synthase subunit 2, 2.5.1.-, Candidate tumor suppressor protein, Decaprenyl pyrophosphate synthetase subunit 2
Gene: PDSS2 Accession Number: HS15203 (MetaCyc)

Molecular Weight: 44.129 kD (from nucleotide sequence)
Gene Class: UNCLASSIFIED

Unification Links: Entrez:AAF97788 , Entrez:AAH39906 , Mint:MINT-6778201 , PhosphoSite:Q86YH6 , PhylomeDB:Q86YH6 , Pride:Q86YH6 , Protein Model Portal:Q86YH6 , SMR:Q86YH6 , String:9606.ENSP00000358033 , UniProt:Q86YH6
Relationship Links: InterPro:IN-FAMILY:IPR000092 , InterPro:IN-FAMILY:IPR008949 , InterPro:IN-FAMILY:IPR017446 , Panther:IN-FAMILY:PTHR12001 , Pfam:IN-FAMILY:PF00348 , Prosite:IN-FAMILY:PS00444 , Prosite:IN-FAMILY:PS00723
References
Appelkvist94: Appelkvist EL, Aberg F, Guan Z, Parmryd I, Dallner G (1994). "Regulation of coenzyme Q biosynthesis." Mol Aspects Med 15 Suppl;s37-46. PMID: 7752843
Mollet07: Mollet J, Giurgea I, Schlemmer D, Dallner G, Chretien D, Delahodde A, Bacq D, de Lonlay P, Munnich A, Rotig A (2007). "Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders." J Clin Invest 117(3);765-72. PMID: 17332895
Rotig00: Rotig A, Appelkvist EL, Geromel V, Chretien D, Kadhom N, Edery P, Lebideau M, Dallner G, Munnich A, Ernster L, Rustin P (2000). "Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency." Lancet 356(9227);391-5. PMID: 10972372
Saiki05: Saiki R, Nagata A, Kainou T, Matsuda H, Kawamukai M (2005). "Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans." FEBS J 272(21);5606-22. PMID: 16262699

Gene-Reaction Schematic: ?

Credits:
Created 22-Feb-2008 by , , SRI International

Caspi R





Report Errors or Provide Feedback
Please cite the following article in publications resulting from the use of MetaCyc: Caspi et al, Nucleic Acids Research 42:D459-D471 2014
Page generated by SRI International Pathway Tools version 18.5 on Mon Mar 2, 2015, biocyc14.