Powder: -20°C for 3 years | In solvent: -80°C for 1 year
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 | Availability | Price/USD | Quantity |
---|---|---|---|
1 mg | In stock | $ 55.00 | |
2 mg | In stock | $ 80.00 | |
5 mg | In stock | $ 163.00 | |
10 mg | In stock | $ 295.00 | |
25 mg | In stock | $ 521.00 | |
50 mg | In stock | $ 755.00 | |
100 mg | In stock | $ 1,060.00 | |
500 mg | In stock | $ 2,120.00 | |
1 mL * 10 mM (in DMSO) | In stock | $ 139.00 |
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 |
Powder: -20°C for 3 years | In solvent: -80°C for 1 year
DMSO: 50 mg/mL (128.37 mM)
You can also refer to dose conversion for different animals. More
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Ibiglustat 1401090-53-6 Metabolism Transferase oral Parkinson's disease Venglustat GZ-402671 inhibit SAR402671 Inhibitor GBA SAR 402671 SAR-402671 GZ 402671 Fabry disease GL-3 levels GZ402671 mutations inhibitor