N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE
- Product Name
- N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE
- CAS No.
- 210110-90-0
- Chemical Name
- N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE
- Synonyms
- NB-DNJ, HCL;Miglustat HCl;(2R,3R,4R,5S)-;N-BUTYL-DNJ, HCL;NB-DNJ hydrochloride;OGT918 hydrochloride;Zavesca hydrochloride;Miglustat HCl (OGT-918;Miglustat, Hydrochloride;N-BUTYLDEOXYNOJIRIMYCIN HCL
- CBNumber
- CB7328886
- Molecular Formula
- C10H22ClNO4
- Formula Weight
- 255.74
- MOL File
- 210110-90-0.mol
N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE Property
- 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
- Hazard Codes
- Xi
- HS Code
- 2933399990
Hazard and Precautionary Statements (GHS)
- Symbol(GHS)
-
- Signal word
- Warning
- Hazard statements
-
H302Harmful if swallowed
H315Causes skin irritation
H319Causes serious eye irritation
H335May cause respiratory irritation
- Precautionary statements
-
P261Avoid breathing dust/fume/gas/mist/vapours/spray.
P305+P351+P338IF IN EYES: Rinse cautiously with water for several minutes. Remove contact lenses, if present and easy to do. Continuerinsing.
N-Bromosuccinimide Price
- Product number
- 21065
- Product name
- N-Butyldeoxynojirimycin (hydrochloride)
- Purity
- ≥98%
- Packaging
- 1mg
- Price
- $37
- Updated
- 2024/03/01
- Product number
- 21065
- Product name
- N-Butyldeoxynojirimycin (hydrochloride)
- Purity
- ≥98%
- Packaging
- 5mg
- Price
- $98
- Updated
- 2024/03/01
- Product number
- 21065
- Product name
- N-Butyldeoxynojirimycin (hydrochloride)
- Purity
- ≥98%
- Packaging
- 10mg
- Price
- $161
- Updated
- 2024/03/01
- Product number
- 21065
- Product name
- N-Butyldeoxynojirimycin (hydrochloride)
- Purity
- ≥98%
- Packaging
- 25mg
- Price
- $353
- Updated
- 2024/03/01
- Product number
- 3117
- Product name
- Miglustathydrochloride
- Packaging
- 10
- Price
- $227
- Updated
- 2021/12/16
N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE Chemical Properties,Usage,Production
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 (0.2?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. |
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
N-BUTYLDEOXYNOJIRIMYCIN, HYDROCHLORIDE Preparation Products And Raw materials
Raw materials
Preparation Products
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