Isofagomine D-Tartrate (CAS 957230-65-8)

Isofagomine D-Tartrate operates as a selective inhibitor of human acid β-glucosidase, playing a pivotal role in the enhancement of lysosomal β-glucosidase activity, particularly noted within mutant N370S Gaucher fibroblasts where it elevates enzyme activity by 2- to 3-fold. This compound engages directly with the enzyme′s catalytic pocket, functioning as a chemical chaperone. Its mechanism of action is not merely inhibitory but also facilitative, as it significantly contributes to the stabilization and proper folding of β-glucosidase. This process, in turn, results in an increase in the functional quantity of the enzyme within the lysosomes, highlighting its critical role in biochemical research focused on understanding enzymatic function and stabilization mechanisms. Through its unique interaction with β-glucosidase, Isofagomine D-Tartrate serves as a key tool in the study of enzyme kinetics and the molecular basis of enzyme dysfunction, offering insights into the intricate balance of enzymatic regulation and its implications for cellular biochemistry.