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Ibiglustat

Catalog No. T4473   CAS 1401090-53-6
Synonyms: 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.

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Ibiglustat Chemical Structure
Ibiglustat, CAS 1401090-53-6
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
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Purity: 99.14%
Purity: 97.97%
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Biological Description
Chemical Properties
Storage & Solubility Information
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

Storage

Powder: -20°C for 3 years | In solvent: -80°C for 1 year

Solubility Information

DMSO: 50 mg/mL (128.37 mM)

TargetMolReferences and Literature

1. Schiffmann R, et al. Fabry disease. Handb Clin Neurol. 2015;132:231-48.

Related compound libraries

This product is contained In the following compound libraries:
Drug Repurposing Compound Library Inhibitor Library Pediatric Drug Library Anti-Metabolism Disease Compound Library CNS-Penetrant Compound Library Bioactive Compound Library ReFRAME Related Library NO PAINS Compound Library Metabolism Compound Library Clinical Compound Library

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Keywords

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

 

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