| Entrez Gene ID |
23590 |
| Official Gene Symbol |
PDSS1 (GeneCards) |
| Full name |
decaprenyl diphosphate synthase subunit 1 |
| Location |
10p12.1 |
| Other ids |
Vega : OTTHUMG00000017844
MIM : 607429
HGNC : HGNC:17759
Ensembl : ENSG00000148459
|
| Other names |
DPS,
SPS,
TPT,
COQ1,
TPRT,
TPT1,
hDPS1,
COQ10D2
|
| 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] |