کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10834439 | 1065890 | 2008 | 8 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis](/preview/png/10834439.png)
چکیده انگلیسی
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.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Molecular Genetics and Metabolism - Volume 94, Issue 2, June 2008, Pages 204-211
Journal: Molecular Genetics and Metabolism - Volume 94, Issue 2, June 2008, Pages 204-211
نویسندگان
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,