| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 10834439 | Molecular Genetics and Metabolism | 2008 | 8 Pages |
Abstract
The imino sugars N-butyldeoxynojirimycin (NB-DNJ, miglustat, Zavescaâ¢) and N-butyldeoxygalactonojirimycin (NB-DGJ) used for SRT inhibit glucosylceramide synthase (GlcCerS) that catalyses the first committed step in glycosphingolipid biosynthesis. We have compared the efficacy and tolerability of NB-DNJ and NB-DGJ in the β-galactosidase knockout mouse. NB-DGJ was better tolerated than NB-DNJ, due to intrinsic gastrointestinal tract dysfunction that was exacerbated by NB-DNJ. However, functional improvement was greatest with NB-DNJ treatment which may potentially be caused by novel anti-inflammatory properties of NB-DNJ.
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Authors
Elena Elliot-Smith, Anneliese O. Speak, Emyr Lloyd-Evans, David A. Smith, Aarnoud C. van der Spoel, Mylvaganam Jeyakumar, Terry D. Butters, Raymond A. Dwek, Alessandra d'Azzo, Frances M. Platt,