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MGP004712

UniProt Annotations

Entry Information
Gene Nameprenyl (decaprenyl) diphosphate synthase, subunit 1
Protein EntryDPS1_HUMAN
UniProt IDQ5T2R2
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=Q5T2R2-1; Sequence=Displayed; Name=2; IsoId=Q5T2R2-2; Sequence=VSP_017101; Note=No experimental confirmation available.; Name=3; IsoId=Q5T2R2-3; Sequence=VSP_017100;
Catalytic Activity(2E,6E)-farnesyl diphosphate + 7 isopentenyl diphosphate = 7 diphosphate + all-trans-decaprenyl diphosphate. {ECO:0000269|PubMed:16262699}.
CofactorName=Mg(2+); Xref=ChEBI:CHEBI:18420; Evidence={ECO:0000250}; Note=Binds 3 Mg(2+) ions per subunit. {ECO:0000250};
DiseaseCoenzyme Q10 deficiency, primary, 2 (COQ10D2) [MIM:614651]: An autosomal recessive multisystem disorder characterized by early-onset deafness, optic atrophy, mild mental retardation, peripheral neuropathy, obesity, livedo reticularis, and cardiac valvulopathy. {ECO:0000269|PubMed:17332895}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionSupplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10. {ECO:0000269|PubMed:16262699}.
PathwayCofactor biosynthesis; ubiquinone biosynthesis.
SimilarityBelongs to the FPP/GGPP synthase family. {ECO:0000305}.
Subcellular LocationMitochondrion {ECO:0000305}.
SubunitHeterotetramer of 2 DPS1/TPRT and 2 DLP1 subunits. {ECO:0000269|PubMed:16262699}.
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