The imino sugarN-butyldeoxynojirimycin is an inhibitor of the ceramide-specific
glucosyltransferase that catalyzes the first step in glycosphingolipid biosynthesis. It results in
extensive glycosphingolipid depletion in cells treated in vitro, without causing toxicity.
However, we currently do not know the degree to which glycosphingolipids can be depleted
in vivo in a mammalian species. We have therefore administeredN-butyldeoxynojirimycin
long term to young mice and have found that glycosphingolipid levels are reduced (50 …