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麦格司他

麦格司他结构式
麦格司他结构式
品牌特惠专场
常用名 麦格司他 英文名 miglustat
CAS号 72599-27-0 分子量 219.278
密度 1.2±0.1 g/cm3 沸点 394.7±42.0 °C at 760 mmHg
分子式 C10H21NO4 熔点 N/A
MSDS 中文版 美版 闪点 215.4±26.5 °C

Development of a cell-based hepatitis C virus infection fluorescent resonance energy transfer assay for high-throughput antiviral compound screening.

Antimicrob. Agents Chemother. 53(10) , 4311-9, (2009)

A major obstacle in the treatment of chronic hepatitis C virus (HCV) infection has been the lack of effective, well-tolerated therapeutics. Notably, the recent development of the HCV cell culture infection system now allows not only for the study of the entir...

Inhibitors of the tick-borne, hemorrhagic fever-associated flaviviruses.

Antimicrob. Agents Chemother. 58(6) , 3206-16, (2014)

No antiviral therapies are available for the tick-borne flaviviruses associated with hemorrhagic fevers: Kyasanur Forest disease virus (KFDV), both classical and the Alkhurma hemorrhagic fever virus (AHFV) subtype, and Omsk hemorrhagic fever virus (OHFV). We ...

Modulation of hepatitis C virus genome replication by glycosphingolipids and four-phosphate adaptor protein 2.

J. Virol. 88(21) , 12276-95, (2014)

Hepatitis C virus (HCV) assembles its replication complex on cytosolic membrane vesicles often clustered in a membranous web (MW). During infection, HCV NS5A protein activates PI4KIIIα enzyme, causing massive production and redistribution of phosphatidylinosi...

Searching for combinations of small-molecule correctors to restore f508del-cystic fibrosis transmembrane conductance regulator function and processing.

J. Pharmacol. Exp. Ther. 350(3) , 624-34, (2014)

The mutated protein F508del-cystic fibrosis transmembrane conductance regulator (CFTR) failed to traffic properly as a result of its retention in the endoplasmic reticulum and functions as a chloride (Cl(-)) channel with abnormal gating and endocytosis. Small...

CNS, lung, and lymph node involvement in Gaucher disease type 3 after 11 years of therapy: clinical, histopathologic, and biochemical findings.

Mol. Genet. Metab. 114(2) , 233-41, (2015)

A Caucasian male with Gaucher disease type 3, treated with continuous enzyme therapy (ET) for 11 years, experienced progressive mesenteric and retroperitoneal lymphadenopathy, lung disease, and neurological involvement leading to death at an age of 12.5 years...

Reduced cerebellar neurodegeneration after combined therapy with cyclodextrin/allopregnanolone and miglustat in NPC1: a mouse model of Niemann-Pick type C1 disease.

J. Neurosci. Res. 93(3) , 433-42, (2015)

Niemann-Pick type C1 (NPC1) disease is a lysosomal storage disease characterized by a deficiency of NPC1 gene function. The malfunction of protein results in a progressive accumulation of lipids in many organs. A combined approach with substrate-reduction the...

Elevation in sphingomyelin synthase activity is associated with increases in amyloid-beta peptide generation.

PLoS ONE 8 , e74016, (2013)

A pathological hallmark of Alzheimer's disease (AD) is the presence of amyloid-beta peptide (Aβ) plaques in the brain. Aβ is derived from a sequential proteolysis of the transmenbrane amyloid precursor protein (APP), a process which is dependent on the distri...

Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin.

Science 276 , 428, (1997)

The glycosphingolipid (GSL) lysosomal storage diseases result from the inheritance of defects in the genes encoding the enzymes required for catabolism of GSLs within lysosomes. A strategy for the treatment of these diseases, based on an inhibitor of GSL bios...

Extensive glycosphingolipid depletion in the liver and lymphoid organs of mice treated with N-butyldeoxynojirimycin.

J. Biol. Chem. 272 , 19365, (1997)

The imino sugar N-butyldeoxynojirimycin is an inhibitor of the ceramide-specific glucosyltransferase that catalyzes the first step in glycosphingolipid biosynthesis. It results in extensive glycosphingolipid depletion in cells treated in vitro, without causin...

N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with impaired gp120 shedding and gp41 exposure.

J. Virol. 70 , 7153, (1996)

The alpha-glucosidase inhibitor N-butyldeoxynojirimycin (NB-DNJ) is an inhibitor of human immunodeficiency virus (HIV) replication and HIV-induced syncytium formation in vitro. Although an NB-DNJ-mediated change in viral envelope N-glycan composition inhibits...