CAS 141206-42-0: N-Butyl-1-deoxygalactonojirimycin

Applications N-(n-Butyl)deoxygalactonojirimycin is an extremely potent and selective a-D-galactosidase inhibitor. A selective inhibitior of GlcCer synthesis. References Paulsen, H., et al.: Chem. Ber., 113, 2601 (1980), Daniel, P.F., et al.: Proc. Int. Carbo. Symp., 13th, Ithaca, NY, Abstr. C43 (1986), Platt, F.M., et al.: J. Biol. Chem., 269, 27108 (1994)

Lucerastat(NBDGJ) is an orally available inhibitor of GCS for Fabry disease by reducing harmful substrates.. GCS is a key enzyme in sphingolipid synthesiss.

Competitive inhibitor of glucosylceramidase (GlcCer), which catalyses the first step in glycosphingolipid (GSL) synthesis. This compound is a promising candidate for substrate reduction therapy in Sandhoff disease, where it decreases the rate of GSL biosynthesis and counterbalances the impaired rate of ganglioside catabolism.

(2R,3S,4R,5S)-1-(But-1-yl)-2-(hydroxymethyl)piperidine-3,4,5-triol