N-Butyldeoxynojirimycin·HCl (CAS 210110-90-0)
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N-Butyldeoxynojirimycin·HCl is a novel inhibitor of UGCG (glucosylceramide synthase) as well as Glucosidase I and II (alpha-glucosidase I and II). The compound may act to inhibit glucosylceramide synthase without ceramide accumulation and cell death. When administered to young mice the compound reduced glycosphingolipid production 50%-70% in the liver and lymphoid organs without any overt pathology. It is a potent inhibitor of HIV virus replication and syncytium formation in vitro. The compound has been observed to restore cAMP-activated chloride currents in epithelial cystic fibrosis cells through prevented interaction among calnexin and delF508-CFTR. In a mice model for Tay Sachs disease, mice exposed to N-Butyldeoxynojirimycin·HCl limited the biosynthesis of GM2 thus preventing accumulation of GM2 in the brain and glycosphingolipids. This action markedly reduced the number of storage neurons and gangliosides per cell. N-Butyldeoxynojirimycin·HCl has also rescued trafficking-deficient F508del-CTFR in human airway epithelial cells via inhibition of ER-glucosidases I and II. Also has broad spectrum antiviral activity.
N-Butyldeoxynojirimycin·HCl (CAS 210110-90-0) References
- Differential effects of glycolipid biosynthesis inhibitors on ceramide-induced cell death in neuroblastoma cells. | Bieberich, E., et al. 1999. J Neurochem. 72: 1040-9. PMID: 10037475
- Targeting glycosylation as a therapeutic approach. | Dwek, RA., et al. 2002. Nat Rev Drug Discov. 1: 65-75. PMID: 12119611
- Ceramide synthesis correlates with the posttranscriptional regulation of the sterol-regulatory element-binding protein. | Worgall, TS., et al. 2004. Arterioscler Thromb Vasc Biol. 24: 943-8. PMID: 15132973
- Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat. | Norez, C., et al. 2006. FEBS Lett. 580: 2081-6. PMID: 16546175
- Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells. | Noël, S., et al. 2008. J Pharmacol Exp Ther. 325: 1016-23. PMID: 18309088
- A role for glycolipid biosynthesis in severe fever with thrombocytopenia syndrome virus entry. | Drake, MJ., et al. 2017. PLoS Pathog. 13: e1006316. PMID: 28388693
- Differential Effects of 2-Hydroxypropyl-Cyclodextrins on Lipid Accumulation in Npc1-Null Cells. | Hoque, S., et al. 2020. Int J Mol Sci. 21: PMID: 32019132
- Gangliosides are essential endosomal receptors for quasi-enveloped and naked hepatitis A virus. | Das, A., et al. 2020. Nat Microbiol. 5: 1069-1078. PMID: 32451473
- The alpha-glucosidase inhibitor N-butyldeoxynojirimycin inhibits human immunodeficiency virus entry at the level of post-CD4 binding. | Fischer, PB., et al. 1995. J Virol. 69: 5791-7. PMID: 7543588
- N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis. | Platt, FM., et al. 1994. J Biol Chem. 269: 8362-5. PMID: 8132559
- N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with impaired gp120 shedding and gp41 exposure. | Fischer, PB., et al. 1996. J Virol. 70: 7153-60. PMID: 8794362
- Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin. | Platt, FM., et al. 1997. Science. 276: 428-31. PMID: 9103204
- Extensive glycosphingolipid depletion in the liver and lymphoid organs of mice treated with N-butyldeoxynojirimycin. | Platt, FM., et al. 1997. J Biol Chem. 272: 19365-72. PMID: 9235935
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
N-Butyldeoxynojirimycin·HCl, 5 mg | sc-201398 | 5 mg | $148.00 | |||
N-Butyldeoxynojirimycin·HCl, 25 mg | sc-201398A | 25 mg | $492.00 | |||
N-Butyldeoxynojirimycin·HCl, 50 mg | sc-201398B | 50 mg | $928.00 |