DHRSX is an oxidoreductase that plays a critical role in dolichol biosynthesis, an essential lipid for N-linked glycosylation 1. The enzyme catalyzes two non-consecutive steps in dolichol synthesis: it first converts polyprenol to polyprenal through NAD+-dependent dehydrogenation, and then reduces dolichal to dolichol using NADPH as a cofactor 1. SRD5A3 catalyzes the intermediate step, reducing polyprenal to dolichal, revealing unexpected complexity in terminal dolichol biosynthesis 1. DHRSX is located on the pseudoautosomal regions of the X and Y chrX|Y, making it subject to unusual inheritance patterns. Missense variants in DHRSX cause congenital disorder of glycosylation type 1DD (DHRSX-CDG), a pseudoautosomal-recessive disease characterized by defects in N-glycosylation 1. Beyond glycosylation, DHRSX may also promote starvation-induced autophagy, though its primary biological role centers on dolichol metabolism 2. DHRSX deficiency impairs N-glycan synthesis and alters polyisoprenoid lipid homeostasis 3. The gene has also been identified as a candidate in nonobstructive azoospermia and appears associated with chemotherapy resistance in pediatric acute lymphoblastic leukemia, though the mechanisms in these contexts remain to be clarified 4, 5.