| 中文名称: | Miglustat | ||||
|---|---|---|---|---|---|
| 英文名称: | Miglustat | ||||
| 别名: | 美格鲁特; 2R,3R,4R,5S)-1-丁基-2-(羟甲基)哌啶-3,4,5-三醇 N-Butyldeoxynojirimycin; NB-DNJ; OGT 918;(2R,3R,4R,5S)-1-Butyl-2-(hydroxymethyl)piperidine-3,4,5-triol | ||||
| CAS No: | 72599-27-0 | 分子式: | C10H21NO4 | 分子量: | 219.28 |
| CAS No: | 72599-27-0 | ||||
| 分子式: | C10H21NO4 | ||||
| 分子量: | 219.28 | ||||
一种葡萄糖神经酰胺合成酶抑制剂，可作用于I型戈谢病。
Miglustat, also known as OGT 918, is a drug developed by Actelion and is used primarily to treat Type I Gaucher disease (GD1). It is marketed under the trade name Zavesca. Miglustat is an imino sugar, a synthetic analogue of D-glucose and a white to off-white crystalline solid that has a bitter taste. The primary pharmacological activity of miglustat is inhibition of the enzyme glucosylceramide synthase, catalyzing the first step in the biosynthesis of glycosphingolipids (GSL), i.e., the formation of glucosylceramide (GlcCer). Reduced formation of GlcCer will lead to decreased biosynthesis of more complex GSL. This therapeutic principle, called substrate reduction therapy (SRT), may be useful in disorders of intracellular (predominantly lysosomal) accumulation of GSL either due to their deficient breakdown or intracellular transport/trafficking. Miglustat exhibits a large volume of distribution and has the capacity to access deep organs such as the brain, bone and lung. (copied from http://en.wikipedia.org/wiki/Miglustat).
本计算器可帮助您计算出特定溶液中溶质的质量、溶液浓度和体积之间的关系，公式为：
质量 (g) = 浓度 (mol/L) x 体积 (L) x 分子量 (g/mol)
摩尔浓度计算公式
用本工具协助配置特定浓度的溶液，使用的计算公式为：
开始浓度 x 开始体积 = 最终浓度 x 最终体积
稀释公式
稀释公式一般简略地表示为：C1V1 = C2V2 ( 输入 输出 )
