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Samples in periodicals archive:
For SRT, N-butyl deoxynojirimycin (Zavesca[R]) is approved as an inhibitor of glucosylceramide syntheses enzyme.
Another interesting class of inhibitors such as celgosivir, n-butyl deoxynojirimycin and N-(n-nonyl) deoxynojirimycin target host alpha-glucosidase enzyme and may offer alternative treatment options (131,132).
In substrate reduction therapy (SRT), the synthesis of glycosphingolipids is reduced by partial inhibition of glucosylceramide (G1cCer) synthase by deoxynojirimycin analogs (5), leading to reduction of the glycosphingolipid pool and eventually to reduction of the substrate load for lysosomal degradation by sphingolipid hydrolases.