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Ibiglustat

Catalog No. T4473Cas No. 1401090-53-6
Alias Venglustat, SAR402671, GZ402671

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.

Ibiglustat

Ibiglustat

Purity: 99.14%
Catalog No. T4473Alias Venglustat, SAR402671, GZ402671Cas No. 1401090-53-6
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 SizePriceAvailabilityQuantity
1 mg$33In Stock
5 mg$98In Stock
10 mg$177In Stock
25 mg$313In Stock
50 mg$453In Stock
100 mg$636In Stock
200 mg$857In Stock
1 mL x 10 mM (in DMSO)$83In Stock
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Purity:99.14%
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Product Introduction

Bioactivity
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].
AliasVenglustat, SAR402671, GZ402671
Chemical Properties
Molecular Weight389.49
FormulaC20H24FN3O2S
Cas No.1401090-53-6
Storage & Solubility Information
StoragePowder: -20°C for 3 years | In solvent: -80°C for 1 year | Shipping with blue ice.
Solubility Information
DMSO: 50 mg/mL (128.37 mM)
Solution Preparation Table
DMSO
1mg5mg10mg50mg
1 mM2.5675 mL12.8373 mL25.6746 mL128.3730 mL
5 mM0.5135 mL2.5675 mL5.1349 mL25.6746 mL
10 mM0.2567 mL1.2837 mL2.5675 mL12.8373 mL
20 mM0.1284 mL0.6419 mL1.2837 mL6.4187 mL
50 mM0.0513 mL0.2567 mL0.5135 mL2.5675 mL
100 mM0.0257 mL0.1284 mL0.2567 mL1.2837 mL

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