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pdss1

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Official Full Name
prenyl (decaprenyl) diphosphate synthase, subunit 1
Background
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.
Synonyms
PDSS1; prenyl (decaprenyl) diphosphate synthase, subunit 1; TPRT, trans prenyltransferase; decaprenyl-diphosphate synthase subunit 1; coenzyme Q1 homolog (yeast); COQ1; TPT; coenzyme Q1 homolog; trans-prenyltransferase 1; trans-prenyltransferase (TPT); p; tprt; wu:fc11f12; wu:fd05d05; zgc:112058; trans-prenyltransferase