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Pack Size | Price | Availability | Quantity |
---|---|---|---|
1 mg | $55 | In Stock | |
2 mg | $80 | In Stock | |
5 mg | $163 | In Stock | |
10 mg | $295 | In Stock | |
25 mg | $521 | In Stock | |
50 mg | $755 | In Stock | |
100 mg | $1,060 | In Stock | |
500 mg | $2,120 | In Stock | |
1 mL x 10 mM (in DMSO) | $139 | 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]. |
Alias | Venglustat, SAR402671, GZ402671 |
Molecular Weight | 389.49 |
Formula | C20H24FN3O2S |
Cas No. | 1401090-53-6 |
Storage | Powder: -20°C for 3 years | In solvent: -80°C for 1 year | |||||||||||||||||||||||||||||||||||
Solubility Information | DMSO: 50 mg/mL (128.37 mM) ![]() | |||||||||||||||||||||||||||||||||||
Solution Preparation Table | ||||||||||||||||||||||||||||||||||||
DMSO
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