Miglustat reduces the biosynthesis of glucosylceramide from ceramide through the inhibition
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چکیده
Miglustat reduces the biosynthesis of glucosylceramide from ceramide through the inhibition of the enzyme glucosylceramide synthase. Glucosylceramide is the first committed step in the synthesis of some 300 to 400 glycosphingolipids. This inhibitory action forms the rationale for substrate reduction therapy in Gaucher disease for which excessive accumulation of glucosylceramide underlies the pathogenesis.
منابع مشابه
N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.
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تاریخ انتشار 2005