UGCG and Gaucher disease: Thus, the N-substituted 1-deoxynojirimycin (DNJ) derivative miglitol (Glyset®, Pfizer, New York, NY, USA), an inhibitor of the intestinal α-glucosidases, is used for glycemic control in the management of type 2 diabetes mellitus [5], whereas N-butyl-DNJ (miglustat; Zavesca®, Actelion Pharmaceuticals Ltd., Allschwil, Switzerland), which inhibits glucosylceramide synthase, is indicated in the treatment of Gaucher disease, a lysosomal storage disorder (LSD) resulting from the malfunctioning of the acid β-glucosidase (β-glucocerebrosidase, GCase) [6].