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Ibiglustat (GZ402671) is a potent and selective Glucosylceramide synthase inhibitor and ceramide glucosyltransferase inhibitor. Ibiglustat blocks the formation of glucosylceramide (GL-1), a key intermediate in the synthesis of GL-3. Ibiglustat is potentially useful for treating Fabry disease. Fabry disease is a rare lysosomal storage disorder, which results in abnormal tissue deposits of a particular fatty substance called globotriaosylceramide (GL-3 or Gb3) throughout the body.

| Pack Size | Price | USA Warehouse | Global Warehouse | Quantity |
|---|---|---|---|---|
| 1 mg | $33 | In Stock | In Stock | |
| 5 mg | $98 | In Stock | In Stock | |
| 10 mg | $177 | In Stock | In Stock | |
| 25 mg | $313 | In Stock | In Stock | |
| 50 mg | $453 | In Stock | In Stock | |
| 100 mg | $636 | In Stock | In Stock | |
| 200 mg | $857 | - | In Stock | |
| 1 mL x 10 mM (in DMSO) | $83 | In Stock | In Stock |
| Description | Ibiglustat (GZ402671) is a potent and selective Glucosylceramide synthase inhibitor and ceramide glucosyltransferase inhibitor. Ibiglustat blocks the formation of glucosylceramide (GL-1), a key intermediate in the synthesis of GL-3. Ibiglustat is potentially useful for treating Fabry disease. Fabry disease is a rare lysosomal storage disorder, which results in abnormal tissue deposits of a particular fatty substance called globotriaosylceramide (GL-3 or Gb3) throughout the body. |
| In vitro | Ibiglustat is in phase II Clinical trials for the treatment of Fabry's disease. Fabry disease, an X-linked disorder of glycosphingolipids that is caused by mutations of the GLA gene that codes for α-galactosidase A, leads to dysfunction of many cell types and includes a systemic vasculopathy [1]. |
| Synonyms | Venglustat, SAR402671, GZ402671 |
| Molecular Weight | 389.49 |
| Formula | C20H24FN3O2S |
| Cas No. | 1401090-53-6 |
| Smiles | CC(C)(NC(=O)O[C@@H]1CN2CCC1CC2)c1csc(n1)-c1ccc(F)cc1 |
| Relative Density. | 1.30 g/cm3 (Predicted) |
| Color | White |
| Appearance | Solid |
| Storage | store at low temperature | Powder: -20°C for 3 years | In solvent: -80°C for 1 year | Shipping with blue ice/Shipping at ambient temperature. | |||||||||||||||||||||||||||||||||||
| Solubility Information | DMSO: 50 mg/mL (128.37 mM), Sonication is recommended. | |||||||||||||||||||||||||||||||||||
| In Vivo Formulation | 10% DMSO+40% PEG300+5% Tween 80+45% Saline: 2 mg/mL (5.13 mM), Sonication is recommended. Please add the solvents sequentially, clarifying the solution as much as possible before adding the next one. Dissolve by heating and/or sonication if necessary. Working solution is recommended to be prepared and used immediately. The formulation provided above is for reference purposes only. In vivo formulations may vary and should be modified based on specific experimental conditions. | |||||||||||||||||||||||||||||||||||
Solution Preparation Table | ||||||||||||||||||||||||||||||||||||
DMSO
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