PDSS1, Official Symbol PDSS1provided by HGNC Official Full Name decaprenyl diphosphate synthase subunit 1provided by HGNC Primary source HGNC:HGNC:17759 See related Ensembl:ENSG00000148459 MIM:607429; AllianceGenome:HGNC:17759 Gene type protein coding RefSeq status REVIEWED Organism Homo sapiens Lineage Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as DPS; SPS; TPT; COQ1; TPRT; COQ1A; TPT 1; 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] Expression Broad expression in duodenum (RPKM 4.4), small intestine (RPKM 3.8) and 24 other tissues See more Orthologs mouse all