| Description | Ibiglustat (L-Malic acid) (Ibiglustat L-Malic acid) is a selective, brain-penetrant, and allosteric inhibitor of glucosylceramide synthase. Ibiglustat (L-Malic acid) can be used in studies about PD Parkinson’s disease, SRT in Fabry’s and Gaucher’s. |
| In vitro | Ibiglustat (L-Malic acid) （1 μM）处理的Fabry病细胞中的GL-3含量接近未处理野生型细胞的生理水平。Ibiglustat (L-Malic acid) 可阻止GL-3的进一步积聚，并改善FD心肌细胞中丰富的GL-3含量[4]。 |
| Synonyms | Ibiglustat L-Malic acid, GZ402671 (L-Malic acid), Venglustat (L-Malic acid), SAR402671 (L-Malic acid) |
| molecular weight | 523.57 |
| Molecular formula | C24H30FN3O7S |
| CAS | 1629063-78-0 |
| Storage | Powder: -20°C for 3 years | In solvent: -80°C for 1 year |
| Solubility | DMSO: 90 mg/mL (171.9 mM) |
| References | 1. Glucosylceramide Synthase Inhibitors. WO 2015089067 A1. 2. Iva Stojkovska, et al. Molecular mechanisms of α-synuclein and GBA1 in Parkinson’s disease. Cell Tissue Res. 2017. 3. Christoph Arenz, et al. Recent advances and novel treatments for sphingolipidoses. Future Med. Chem. (2017) 9(14), 1687–1700. 4. Itier JM, et al. Effective clearance of GL-3 in a human iPSC-derived cardiomyocyte model of Fabry disease. J Inherit Metab Dis. 2014 Nov;37(6):1013-22. |