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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 | Availability | Quantity |
|---|---|---|---|
| 1 mg | $33 | In Stock | |
| 5 mg | $98 | In Stock | |
| 10 mg | $177 | In Stock | |
| 25 mg | $313 | In Stock | |
| 50 mg | $453 | In Stock | |
| 100 mg | $636 | In Stock | |
| 200 mg | $857 | In Stock | |
| 1 mL x 10 mM (in DMSO) | $83 | 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 | 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. | |||||||||||||||||||||||||||||||||||
Solution Preparation Table | ||||||||||||||||||||||||||||||||||||
DMSO
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