Miglustat HCl | Alpha-glucosidase inhibitor

CAS:
210110-90-0
Catalog Number:
10-2413
Activity:
Alpha-glucosidase inhibitor
Chemical Name:
N-Butyldeoxynojirimycin HCl
Alternate Names:
NB-DNJ
Molecular Weight:
255.74
Molecular Formula:
C10H21NO4 • HCL
Solubility:
Soluble in DMSO (up to 20 mg/ml) or in Water (up to 20 mg/ml)
Physical Properties:
White solid
Purity:
98% by TLC
NMR (Conforms)
Storage Temperature:
-20°C
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.
Shipping Code:
RT

Available Options

Size :PriceQuantity 
5 mg$80.00
25 mg$300.00

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

References/Citations:

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

CAS:
210110-90-0
Catalog Number:
10-2413
Activity:
Alpha-glucosidase inhibitor
Chemical Name:
N-Butyldeoxynojirimycin HCl
Alternate Names:
NB-DNJ
Molecular Weight:
255.74
Molecular Formula:
C10H21NO4 • HCL
Solubility:
Soluble in DMSO (up to 20 mg/ml) or in Water (up to 20 mg/ml)
Physical Properties:
White solid
Purity:
98% by TLC
NMR (Conforms)
Storage Temperature:
-20°C
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.
Shipping Code:
RT

Safety Data Sheet:

N/A

Product Data Sheet:

Materials provided by Focus Biomolecules are for laboratory research use only and are not intended for human or veterinary applications. Please note that we do not sell to individuals and that all orders placed by non-research organizations will incur a $20 restocking/refund fee

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