Biochemistry of glycosphingolipid storage disorders: implications for therapeutic intervention
نویسندگان
چکیده
منابع مشابه
Miglustat: substrate reduction therapy for glycosphingolipid storage disorders
10.1586/14750708.2.4.569 © 2 Miglustat (N-butyldeoxynojirimycin) is a potent inhibitor of ceramide-specific glucosyltransferase, the enzyme that catalyses the first committed step in glycosphingolipid biosynthesis. Inhibition of glycosphingolipid synthesis is a potential strategy for the treatment of a number of lysosomal storage disorders that result in the accumulation of glycosphingolipids w...
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ژورنال
عنوان ژورنال: Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences
سال: 2003
ISSN: 0962-8436,1471-2970
DOI: 10.1098/rstb.2003.1273