Solubility: Soluble in DMSO (up to 20 mg/ml) or in Water (up to 20 mg/ml).
Storage: -20°C
Stability: ≥ 1 year.
Applications
a-Glucosidase inhibitor
Functions
Orally active ceramide-specific glycosyltransferase and α-glucosidase I and II inhibitor. Rescues trafficking-deficient F508del-CFTR in human airway epithelial cells via inhibition of ER α-glucosidases I and II. Pharmacological chaperone for glucocerebrosidase degradation. Clinically useful agent for Gaucher disease type 1. Stabilizes neurological disorders in Niemann-Pick disease type C.
Application Procedures
First dissolved in DMSO (up to 20 mg/ml) or in Water (up to 20 mg/ml), then diluted to aqueous buffer. Solutions in DMSO or distilled water may be stored at -20°C for up to 1 month.
