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Miglustat  分析标准品

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包装规格：20mg in glass bottle
产品描述：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.
保存条件：2-8°C

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