Isofagomine Induced Stabilization of Glucocerebrosidase
نویسندگان
چکیده
منابع مشابه
Stabilization of Glucocerebrosidase by Active Site Occupancy
Glucocerebrosidase (GBA) is a lysosomal β-glucosidase that degrades glucosylceramide. Its deficiency results in Gaucher disease (GD). We examined the effects of active site occupancy of GBA on its structural stability. For this, we made use of cyclophellitol-derived activity-based probes (ABPs) that bind irreversibly to the catalytic nucleophile (E340), and for comparison, we used the potent re...
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Gaucher disease is an autosomal recessive lysosomal storage disorder caused by the deficient activity of glucocerebrosidase. Accumulation of glucosylceramide, primarily in the lysosomes of cells of the reticuloendothelial system, leads to hepatosplenomegaly, anemia and skeletal lesions in type I disease, and neurologic manifestations in types II and III disease. We report herein the identificat...
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Glucocerebrosidase (GCase) functions as a lysosomal enzyme and its mutations are known to be related to many neurodegenerative diseases, including Gaucher's disease (GD), Parkinson's disease (PD), and Dementia with Lewy Bodies (DLB). However, there is little information about the role of GCase in the pathogenesis of Alzheimer's disease (AD). Here we demonstrate that GCase protein levels and enz...
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N-(10-Chloro-9-anthracenemethyl)isofagomine 5 and N-(10-chloro-9-anthracenemethyl)-1-deoxynojirimycin 6 were prepared, and their inhibition of almond β-glucosidase was measured. The isofagomine derivative 5 was found to be a potent inhibitor, while the 1-deoxynojirimycin derivative 6 displayed no inhibition at the concentrations investigated. Fluorescence spectroscopy of 5 with almond β-glucosi...
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شناسایی مخاطرات در واحد 400 پالایشگاه گاز پارسیان. در این پروزه با بکارگیری از تکنیک hazop به شناسا یی مخاطرات ، انحرافات ممکن و در صورت لزوم ارایه راهکارهای مناسب جهت افزایش ایمنی فرا یند پرداخته میگردد. شرایط عملیاتی مخاطره آمیز نظیر فشار و دمای بالا و وجود ترکیبات مختلف سمی و قابل انفجار در واحدهای پالایش گاز، ضرورت توجه به موارد ایمنی در این چنین واحدهایی را مشخص می سازد. مطالعه hazop یک ر...
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ژورنال
عنوان ژورنال: ChemBioChem
سال: 2008
ISSN: 1439-4227,1439-7633
DOI: 10.1002/cbic.200800249