产品说明书
Miglustat HCl
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同义名 : | OGT 918 hydrochloride;N-Butyldeoxynojirimycin hydrochloride;NB-DNJ;Miglustat;N-Butyldeoxynojirimycin (hydrochloride);NB-DNJ hydrochloride;Miglustat hydrochloride |
| CAS号 : | 210110-90-0 | |
| 货号 : | A968121 | |
| 分子式 : | C10H22ClNO4 | |
| 纯度 : | 99%+ | |
| 分子量 : | 255.74 | |
| MDL号 : | MFCD00269940 | |
| 存储条件： |
粉末 Inert atmosphere,Room Temperature 液体 -20°C:3-6个月-80°C:12个月 |
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| 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) |
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| 动物实验配方： |
| 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]. | ||
| 实验方案 | |||
|---|---|---|---|
| 1mg | 5mg | 10mg | |
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1 mM 5 mM 10 mM |
3.91mL 0.78mL 0.39mL |
19.55mL 3.91mL 1.96mL |
39.10mL 7.82mL 3.91mL |
| 参考文献 |
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