Protein Name | decaprenyl diphosphate synthase subunit 1 |
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Organism | Homo sapiens |
Gene ID | 23590 |
Gene Symbol | PDSS1 |
UniProt | Q5T2R2 (DPS1_HUMAN) |
Relationships |
Total Number of functionally related compound(s) :
74
Total Number of Articles : 47 |
Description |
decaprenyl diphosphate synthase subunit 1 |
Gene Summary |
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. [provided by RefSeq, Jul 2008] |
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