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n-Butyldeoxynojirimycin, hydrochloride

Product Namen-Butyldeoxynojirimycin, hydrochloride
CAS210110-90-0
MFC10H22ClNO4
MW255.74
EINECS
MOL File210110-90-0.mol

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

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.

in vivo

Miglustat (0.2mg/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:	0.2mg/kg
Administration:	Oral administration; once
Result:	Was able to rescue synaptic plasticity deficits, to restore ERKs activation and to counteract hyperexcitability.

storage

Desiccate at -20°C

References

[1] F. PLATT. N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.[J]. The Journal of Biological Chemistry, 1994, 106 1: 8362-8365. DOI:10.1016/s0021-9258(17)37202-2
[2] SABRINA NOËL. Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.[J]. Journal of Pharmacology and Experimental Therapeutics, 2008, 325 3: 1016-1023. DOI:10.1124/jpet.107.135582
[3] OLGA ABIAN*. Therapeutic Strategies for Gaucher Disease: Miglustat (NB-DNJ) as a Pharmacological Chaperone for Glucocerebrosidase and the Different Thermostability of Velaglucerase Alfa and Imiglucerase[J]. Molecular Pharmaceutics, 2011, 8 6: 2390-2397. DOI:10.1021/mp200313e
[4] CHRISTINE SERRATRICE Jacques S Laure Swiader. Switching from imiglucerase to miglustat for the treatment of French patients with Gaucher disease type 1: a case series.[J]. Journal of Medical Case Reports, 2015, 9: 146. DOI:10.1186/s13256-015-0617-5
[5] PARVANEH KARIMZADEH. Effects of miglustat on stabilization of neurological disorder in niemann-pick disease type C: Iranian pediatric case series.[J]. Journal of Child Neurology, 2013, 28 12: 1599-1606. DOI:10.1177/0883073812464526

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