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首页 / 抑制剂/激动剂 / 神经信号通路 / 葡萄糖神经酰胺合成酶 / Miglustat HCl

Miglustat HCl {[allProObj[0].p_purity_real_show]}

货号：A968121 同义名： OGT 918 hydrochloride;N-Butyldeoxynojirimycin hydrochloride

Miglustat HCl is an inhibitor of glucosylceramide synthase, primarily to treat Type I Gaucher disease (GD1).

Miglustat HCl 化学结构 CAS号：210110-90-0
Miglustat HCl 化学结构
CAS号：210110-90-0
Miglustat HCl 3D分子结构
CAS号：210110-90-0
Miglustat HCl 化学结构 CAS号：210110-90-0
Miglustat HCl 3D分子结构 CAS号：210110-90-0
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Miglustat HCl 纯度/质量文件 产品仅供科研

货号：A968121 标准纯度： {[allProObj[0].p_purity_real_show]}
批次查询： 批次纯度:

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Miglustat HCl 生物活性

描述 Gaucher disease (GD) is an inherited lipidstorage disorder caused by deficiency of glucocerebrosidase (GlcCerase) activity and characterized by intralysosomal accumulation of glucocerebroside (glucosylceramide; GlcCer) that leads to dysfunction in multiple organ systems. Miglustat is an iminosugar that reversibly inhibits glucosylceramide synthase and reduces GlcCer biosynthesis[3]. The addition of 10 μM miglustat to COS-7 cell medium leads to 1.3-, 2.1-, 2.3-, 3.6-, and 9.9-fold increase in the activity of S364R, wild-type, N370S, V15M, and M123T GC (β-glucosidase), respectively. These results suggest that miglustat, in addition to the inhibitory effect on CSG (glucosyltransferase), also works as a "chemical chaperone", increasing the activity of acid beta-glucosidase of wild-type and several GC mutated proteins, including the most frequent N370S mutation[4].
作用机制 Miglustat binds at the active site[4].

Miglustat HCl 参考文献

[1]Abian O, Alfonso P, et al. 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. 2011 Dec 5;8(6):2390-7.

[2]Platt FM, Neises GR, et al. N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis. J Biol Chem. 1994 Mar 18;269(11):8362-5.

[3]Abian O, Alfonso P, et al. 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. 2011 Dec 5;8(6):2390-7

[4]Alfonso P, Pampín S, Estrada J, et al. Miglustat (NB-DNJ) works as a chaperone for mutated acid beta-glucosidase in cells transfected with several Gaucher disease mutations. Blood Cells Mol Dis. 2005 Sep-Oct;35(2):268-76

Miglustat HCl 实验方案

计算器
存储液制备 1mg 5mg 10mg

1 mM

5 mM

10 mM

3.91mL

0.78mL

0.39mL

19.55mL

3.91mL

1.96mL

39.10mL

7.82mL

3.91mL

Miglustat HCl 技术信息

CAS号210110-90-0
分子式C10H22ClNO4
分子量 255.74
别名 OGT 918 hydrochloride;N-Butyldeoxynojirimycin hydrochloride;NB-DNJ;Miglustat;N-Butyldeoxynojirimycin (hydrochloride);NB-DNJ hydrochloride;Miglustat hydrochloride
运输蓝冰
存储条件

液体 -20°C:3-6个月-80°C:12个月

粉末 Inert atmosphere,Room Temperature
溶解度

DMSO: 65 mg/mL(254.16 mM)，配合低频超声助溶，注意：DMSO长时间开封后，会吸水并导致溶解能力下降，请避免使用长期开封的DMSO

H2O: 30 mg/mL(117.31 mM)
动物实验配方

Tags: Miglustat | 210110-90-0 | N-Butyldeoxynojirimycin | NB-DNJ | OGT 918 | OGT918 | OGT-918 | Glucosylceramide Synthase Inhibitor | Neuronal Signaling | Glucosylceramide Synthase (GCS) | 仅供科研使用 | AmBeed-信号通路专用抑制剂 | Miglustat HCl 纯度/质量文件 | Miglustat HCl 生物活性 | Miglustat HCl 参考文献 | Miglustat HCl 实验方案 | Miglustat HCl 技术信息

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