Ibiglustat

Ibiglustat is an inhibitor of glucosylceramide (GlcCer) synthase. Dietary administration of ibiglustat (0.03% w/w) reduces globotriaosylceramide (Gb3) and lyso-Gb3 accumulation in a variety of tissues, including the kidney, heart, and brain, in the Gla-/- mouse model of Fabry disease. It reduces the latency to paw withdrawal in the hot plate test in the same model, indicating reduced peripheral neuropathy. Ibiglustat (0.03% w/w in the diet) reduces gliosis, delays the onset of ataxia, and increases lifespan in the transgenic 4L,C* mouse model of neuronopathic Gaucher disease.Formal Name: N-[1-[2-(4-fluorophenyl)-4-thiazolyl]-1-methylethyl]-carbamic acid, (3S)-1-azabicyclo[2.2.2]oct-3-yl ester. CAS Number: 1401090-53-6. Synonyms: Genz-682452, GZ/SAR402671, Venglustat. Molecular Formula: C20H24FN3O2S. Formula Weight: 389.5. Purity: >98%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMF: 30 mg/ml, DMSO: 25 mg/ml, Ethanol: 30 mg/ml, Ethanol:PBS (pH 7.2) (1:7): 0.1 mg/ml. lambdamax: 293 nm. SMILES: CC(C)(NC(O[C@@H]1CN2CCC1CC2)=O)C3=CSC(C4=CC=C(F)C=C4)=N3. InChi Code: InChI=1S/C20H24FN3O2S/c1-20(2,17-12-27-18(22-17)14-3-5-15(21)6-4-14)23-19(25)26-16-11-24-9-7-13(16)8-10-24/h3-6,12-13,16H,7-11H2,1-2H3,(H,23,25)/t16-/m1/s1. InChi Key: YFHRCLAKZBDRHN-MRXNPFEDSA-N.