Isofagomine (D-tartrate)

Isofagomine (D-tartrate) is a competitive inhibitor of human lysosomal beta-glucosidase (Ki = 0.016-0.025 µM, IC50 = 0.06 µM). By interacting with the catalytic pocket of beta-glucosidase it acts as a chemical chaperone that increases the amount of beta-glucosidase by stabilizing and/or promoting the folding of the enzyme. Isofagomine (D-tartrate) has been shown to increase lysosomal beta-glucosidase activity by 2- to 3-fold in mutant N370S Gaucher fibroblasts. This compound has been studied in the context of Gaucher disease, a lysosomal storage disorder resulting from substantial deficiency of beta-glucosidase and recently identified as a parkinsonism risk factor.Formal Name: 5R-(hydroxymethyl)-3R,4R-piperidinediol, mono 2S,3S-dihydroxybutanedioate. CAS Number: 957230-65-8. Molecular Formula: C6H13NO3 . C4H6O6. Formula Weight: 297.3. Purity: >95%. Formulation: (Request formulation change), A solution in ethanol:water (1:1). Solubility: DMSO: 2 mg/ml, PBS (pH 7.2): 5 mg/ml. SMILES: O[C@@H]1[C@@H](CO)CNC[C@H]1O.O[C@H](C(O)=O)[C@H](O)C(O)=O. InChi Code: InChI=1S/C6H13NO3.C4H6O6/c8-3-4-1-7-2-5(9)6(4)10,5-1(3(7)8)2(6)4(9)10/h4-10H,1-3H2,1-2,5-6H,(H,7,8)(H,9,10)/t4-,5-,6-,/m1./s1. InChi Key: ULBPPCHRAVUQMC-RWOHWRPJSA-N.