Isofagomine (D-tartrate) is a competitive inhibitor of human lysosomal β-glucosidase (Ki = 0.016-0.025uM; IC50 = 0.06uM).1 By interacting with the catalytic pocket of β-glucosidase it acts as a chemical chaperone that increases the amount of β-glucosidase by stabilizing and/or promoting the folding of the enzyme.2 Isofagomine (D-tartrate) has been shown to increase lysosomal β-glucosidase activity by 2 to 3-fold in mutant N370S Gaucher fibroblasts.1 This compound has been studied in the context of Gaucher disease, a lysosomal storage disorder resulting from substantial deficiency of β-glucosidase and recently identified as a parkinsonism risk factor.2
Usage:Isofagomine (D-tartrate) is supplied as a crystalline solid. A stock solution may be made by dissolving the isofagomine (D-tartrate) in the solvent of choice. Isofagomine (D-tartrate) is soluble in DMSO at a concentration of approximately 2mg/ml. Further dilutions of the stock solution into aqueous buffers or isotonic saline should be made prior to performing biological experiments. Ensure that the residual amount of organic solvent is insignificant, since organic solvents may have physiological effects at low concentrations. Organic solvent-free aqueous solutions of isofagomine (D-tartrate) can be prepared by directly dissolving the crystalline solid in aqueous buffers. The solubility of isofagomine (D-tartrate) in PBS, pH 7.2, is approximately 5mg/ml. We do not recommend storing the aqueous solution for more than one day.
Storage and Stability:May be stored at RT for short-term only. Long-term storage is recommended at -20°C.. Stable for 6 months after receipt. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Store under inert atmosphere.
