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DPS / PDSS1 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.
Gene Name: | prenyl (decaprenyl) diphosphate synthase, subunit 1 |
Synonyms: | PDSS1, DPS, COQ10D2, RP13-16H11.3, SPS, TPRT, TPT 1, Trans-prenyltransferase, Trans-prenyltransferase (TPT), Trans-prenyltransferase 1, Coenzyme Q1 homolog, COQ1, DPS1, HDPS1, TPT |
Target Sequences: | NM_014317 NP_055132.2 Q5T2R2 |
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