Miglustat HCl (N-Butyldeoxynojirimycin), a-Glucosidase inhibitor (TBI2413)

Description

Product Details

Formal Name: N-Butyldeoxynojirimycin HCl

Alternate Names: NB-DNJ

Molecular Formula: C₁₀H₂₁NO₄ HCl

Formula Weight: 255.74

CAS Number: 210110-90-0

Purity: >98%

Formulation: powder

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.