N-Butyldeoxynojirimycin has been used:in the inhibition of glycolipid synthesis in neuroblastoma cellsin the inhibition the ceramide-specific glycosyltransferase in hepatocytesin the inhibition of ?-glucosidase (GBA2) using fluorescence- activity assay in human embryonic kidney (HEK293) cells.
Biochem/physiol Actions
N-Butyldeoxynojirimycin is an inhibitor of glucosyltransferase and ?-glucosidases. N-Butyldeoxynojirimycin, also known as misglustat, reduces glycolipid levels by substrate reduction therapy (SRT) and is effectively used for the treatment of glycosphingolipid lysosomal storage disorder, Gaucher disease.
α-glucosidase Inhibitor
General description
N-Butyldeoxynojirimycin is an alkylated product of imino sugar deoxynojirimycin.