LMPD Database

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LMP005824

Record overview

LMPD IDLMP005824
Gene ID23590
SpeciesHomo sapiens (Human)
Gene Nameprenyl (decaprenyl) diphosphate synthase, subunit 1
Gene SymbolPDSS1
SynonymsCOQ1; COQ10D2; DPS; SPS; TPRT; TPT; TPT 1; hDPS1
Alternate namesdecaprenyl-diphosphate synthase subunit 1; coenzyme Q1 homolog; trans-prenyltransferase 1; trans-prenyltransferase (TPT); polyprenyl pyrophosphate synthetase; decaprenyl pyrophosphate synthase subunit 1; subunit 1 of decaprenyl diphosphate synthase; all-trans-decaprenyl-diphosphate synthase subunit 1
Chromosome10
Map Location10p12.1
EC Number2.5.1.91
SummaryThe 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. Defects in this gene are a cause of coenzyme Q10 deficiency. [provided by RefSeq, Jul 2008]
OrthologsView orthologs and multiple alignments for PDSS1

Proteins

decaprenyl-diphosphate synthase subunit 1
Refseq ID:NP_055132
Protein GI:50659086
UniProt ID:Q5T2R2
mRNA ID:NM_014317
Length:415
RefSeq Status:REVIEWED
MASRWWRWRRGCSWKPAARSPGPGSPGRAGPLGPSAAAEVRAQVHRRKGLDLSQIPYINLVKHLTSACPNVCRISRFHHTTPDSKTHSGEKYTDPFKLGW
RDLKGLYEDIRKELLISTSELKEMSEYYFDGKGKAFRPIIVALMARACNIHHNNSRHVQASQRAIALIAEMIHTASLVHDDVIDDASSRRGKHTVNKIWG
EKKAVLAGDLILSAASIALARIGNTTVISILTQVIEDLVRGEFLQLGSKENENERFAHYLEKTFKKTASLIANSCKAVSVLGCPDPVVHEIAYQYGKNVG
IAFQLIDDVLDFTSCSDQMGKPTSADLKLGLATGPVLFACQQFPEMNAMIMRRFSLPGDVDRARQYVLQSDGVQQTTYLAQQYCHEAIREISKLRPSPER
DALIQLSEIVLTRDK