NUS1 (dehydrodolichyl diphosphate synthase subunit) is a critical component of the dolichol biosynthesis pathway. As a subunit of the dehydrodolichyl diphosphate synthase (DDS) complex with DHDDS, NUS1 catalyzes the condensation of isopentenyl pyrophosphate molecules to synthesize dehydrodolichyl diphosphate, a precursor for dolichol phosphate production 12345. Dolichol phosphate serves as an essential sugar carrier in N-glycosylation within the endoplasmic reticulum, facilitating protein glycosylation and stability of nascent proteins including NPC2, which regulates LDL-derived cholesterol trafficking 123. Additionally, NUS1 acts as a specific receptor for Nogo-B, a neural and cardiovascular regulator 6. NUS1 mutations cause developmental and epileptic encephalopathies (DEE) and progressive myoclonus epilepsy, typically presenting with de novo variants 789. Beyond epilepsy, NUS1-related disorders feature complex movement disorders with myoclonus (68.3% of cases), dystonia, ataxia, and parkinsonism in 87.8% of affected individuals 9. NUS1 is also implicated as a risk gene for Parkinson's disease 1011, with evidence suggesting cholesterol accumulation and dopaminergic neurodegeneration underlie PD pathogenesis 11. In cancer biology, NUS1 loss promotes oxidative stress and ferroptosis sensitivity, inhibiting prostate cancer growth 12.