| Identification | Back Directory | [Name]
N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE | [CAS]
210110-90-0 | [Synonyms]
NB-DNJ, HCL
Miglustat HCl
(2R,3R,4R,5S)-
N-BUTYL-DNJ, HCL
Miglustat, Hydrochloride
N-BUTYLDEOXYNOJIRIMYCIN HCL
N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE
N-(N-BUTYL)DEOXYNOJIRIMYCIN, HYDROCHLORIDE
N-(n-Butyl)-1-deoxynojirimycin hydrochloride min. 99%
Miglustat hydrochloride, N-(n-Butyl)deoxynojirimycin hydrochloride
(2R,3R,4R,5S)-1-Butyl-2-(hydroxymethyl)piperidine-3,4,5-triol hydrochloride
(2R,3R,4R,5S)-1-Butyl-2-(hydroxymethyl)-,3,4,5-Piperidinetriol Hydrochloride
Miglustat HCl, NB-DNJ HCl, (2R,3R,4R,5S)-1-Butyl-2-(hydroxymethyl)piperidine-3,4,5-triol | [Molecular Formula]
C10H22ClNO4 | [MDL Number]
MFCD00269940 | [MOL File]
210110-90-0.mol | [Molecular Weight]
255.74 |
| Chemical Properties | Back Directory | [Appearance]
White Crystals | [Melting point ]
169-172°C | [storage temp. ]
Desiccate at -20°C | [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 Information | Back Directory | [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. | [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 | [in vivo]
Miglustat (0.2?mg/kg; oral administration; once) hydrochloride is able to rescue synaptic plasticity deficits, to restore ERKs activation and to counteract hyperexcitability[2]. | Animal Model: | NPC1?/? mice[1] | | Dosage: | 0.2?mg/kg | | Administration: | Oral administration; once | | Result: | Was able to rescue synaptic plasticity deficits, to restore ERKs activation and to counteract hyperexcitability.
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| [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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