N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE Chemical Properties

Melting point:

169-172°C

storage temp. 

Inert atmosphere,Room Temperature

solubility 

Soluble in DMSO (up to 20 mg/ml) or in Water (up to 20 mg/ml)

form 

White to off-white solid.

color 

White

Stability:

Stable for 1 year from date of purchase as supplied. Solutions in DMSO or distilled water may be stored at -20°C for up to 1 month.

Safety Information

Hazard Codes 

Xi

HS Code 

2933399990

N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE Usage And Synthesis

Description

Miglustat HCl (210110-90-0) is an orally active ceramide-specific glycosyltransferase and α-glucosidase I and II inhibitor.1 Rescues trafficking-deficient F508del-CFTR in human airway epithelial cells via inhibition of ER α-glucosidases I and II.2 Pharmacological chaperone for glucocerebrosidase degradation.3 Clinically useful agent for Gaucher disease type 1.4 Stabilizes neurological disorders in Niemann-Pick disease type C.5

Chemical Properties

White Crystals

Uses

An inhibitor of a-glucosidase 1 as well as being an inhibitor of HIV cytopathicity. N-Butyldeoxynojirimycin has recently been used to retard lipid storage in the central nervous system of an animal m odel of Tay-Sachs disease. 1-Deoxynojirimycin derivatives as selective inhibitors of glucosylceramide metabolism in man.

storage

Desiccate at -20°C

References

1) Platt et al. (1994), N-butyldeoxynojirimycin is a novel inhibitor if glycolipid biosynthesis; J. Biol. Chem., 269 8362 2) Noel et al. (2008), Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis; J. Pharmacol. Exp. Ther., 325 1016 3) Abian et al. (2011), Therapeutic strategies for Gaucher disease: miglustat (NB-DNJ) as a pharmacological chaperone for glucocerebrosidase and the different thermostability of velaglucerase alfa and imiglucerase; Mol. Pharm., 8 2390 4) Serratrice et al. (2015), Switching from imiglucerase to miglustat for the treatment of French patients with Gaucher disease type 1: a case series; J. Med. Case Rep., 9 146 5) Karimzadeh (2013), Effects of miglustat on stabilization of neurological disorder in niemann-pick disease type C: Iranian pediatric case series; J. Child Neurol., 28 1599

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