NUS1 (Nuclear Undecaprenyl Pyrophosphate Synthase 1 Homolog)

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NUS1 (Nuclear Undecaprenyl Pyrophosphate Synthase 1 Homolog) encodes a subunit of dehydrodiphosphate diphosphate synthase (DHDDS). NUS1, together with DHDDS, is essential for protein glycosylation and plays an important role in the regulation of newborn lysosomal NPC2 proteins. Both NUS1 and DHDDS subunits contribute to enzyme activity by condensing multiple copies of isopentenyl pyrophosphate (IPP) to farnesyl pyrophosphate (FPP) to produce dehydrodiphthaloyl diphosphate (Dedol-PP), a precursor of polyhydrophenol phosphate, which is used as a sugar carrier in protein glycosylation, endoplasmic reticulum (ER). NUS1 is associated with autosomal dominant development and epileptic encephalopathy.

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