Search Thermo Fisher Scientific
Search Thermo Fisher Scientific
Peptide sequence: GEFLQLGSKE NENERFAHYL EKTFKKTASL IANSCKAVSV LGCPDPVVHE
Sequence homology: Cow: 100%; Dog: 100%; Guinea Pig: 86%; Horse: 100%; Human: 100%; Mouse: 100%; Pig: 100%; Rabbit: 100%; Rat: 100%; Zebrafish: 93%
PDSS1 is an enzyme that elongates the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. PDSS1 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 PDSS1 gene are a cause of coenzyme Q10 deficiency.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. Defects in this gene are a cause of coenzyme Q10 deficiency.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: All trans-polyprenyl-diphosphate synthase PDSS1; All-trans-decaprenyl-diphosphate synthase subunit 1; coenzyme Q1 homolog; Decaprenyl pyrophosphate synthase subunit 1; Decaprenyl-diphosphate synthase subunit 1; MGC70953; polyprenyl pyrophosphate synthetase; RP13-16H11.3; Solanesyl-diphosphate synthase subunit 1; subunit 1 of decaprenyl diphosphate synthase; TPT 1; trans-prenyltransferase (TPT); Trans-prenyltransferase 1
Gene Aliases: COQ1; COQ10D2; DPS; DPS1; hDPS1; PDSS1; SPS; TPRT; TPT; TPT 1
UniProt ID: (Human) Q5T2R2
Entrez Gene ID: (Human) 23590
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