Entrez gene ID | | 23590 |
Official gene symbol | | PDSS1 |
Full name | | prenyl (decaprenyl) diphosphate synthase, subunit 1 |
Aliases | | ,COQ1,DPS,MGC70953,RP13-16H11.3,SPS,TPRT,TPT,hDPS1, |
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] |
Location | | Chromosome: 10 Locus: 10p12.1 |
Gene position | | 26986595 - 27035727 Map Viewer |
Gene orientation | | plus |
Gene size | | 49133 bp |
Gene sequence |
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OMIM ID | | 607429 |
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