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| | N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE Basic information |
| Product Name: | N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE | | Synonyms: | Miglustat, Hydrochloride;(2R,3R,4R,5S)-1-Butyl-2-(hydroxymethyl)-,3,4,5-Piperidinetriol Hydrochloride;N-BUTYLDEOXYNOJIRIMYCIN HCL;N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE;N-BUTYL-DNJ, HCL;NB-DNJ, HCL;N-(N-BUTYL)DEOXYNOJIRIMYCIN, HYDROCHLORIDE;(2R,3R,4R,5S)-1-Butyl-2-(hydroxymethyl)piperidine-3,4,5-triol hydrochloride | | CAS: | 210110-90-0 | | MF: | C10H22ClNO4 | | MW: | 255.74 | | EINECS: | | | Product Categories: | Glycosidase Inhibitors;Intermediates & Fine Chemicals;Pharmaceuticals;All Inhibitors | | Mol File: | 210110-90-0.mol |  |
| | 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. |
| 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 |
| | N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE Preparation Products And Raw materials |
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