Dehydrodolichyl diphosphate synthase subunit (DHDDS)

Dehydrodolichyl diphosphate synthase subunit (DHDDS) is a highly conserved enzyme and the catalytic core of the human cis-prenyltransferase complex. DHDDS catalyzes cis-prenyl chain elongation by joining isopentenyl diphosphate and farnesyl diphosphate, producing the polyisoprenoid backbone of dolichol, a lipid essential for protein N-glycosylation in the ER. The enzymatic activity and substrate specificity require heteromerization with the Nogo-B receptor (NgBR). DHDDS mutations disrupt dolichol synthesis and are causally associated with diseases like autosomal recessive retinitis pigmentosa and developmental epileptic encephalopathies, emphasizing its central role in human metabolism and disease.

Other names

Dehydrodolichyl diphosphate synthaseDHDDSHDSCITCis-isoprenyltransferaseCis-prenyltransferase subunit hCITDSRP59hCITEpididymis tissue protein Li 189mCPTDEDSMdedol-PP synthase

not applicable (no approved drugs); theoretical mechanism would be enzyme inhibition, blocking dolichol synthesis