Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease - Test2 - elhamkhani - TriplyDB

Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease

Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease

http://www.wikidata.org/entity/Q27646926

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Molecular basis of reduced glucosylceramidase activity in the most common Gaucher disease mutant, N370S Less Is More: Substrate Reduction Therapy for Lysosomal Storage Disorders Acid beta-glucosidase: insights from structural analysis and relevance to Gaucher disease therapy Crystal Structure of the Salmonella enterica Serovar Typhimurium Virulence Factor SrfJ, a Glycoside Hydrolase Family Enzyme Cyclodextrin-mediated crystallization of acid β-glucosidase in complex with amphiphilic bicyclic nojirimycin analogues Pharmacological chaperones for human -N-acetylgalactosaminidase Binding of 3,4,5,6-Tetrahydroxyazepanes to the Acid-β-glucosidase Active Site: Implications for Pharmacological Chaperone Design for Gaucher Disease Structural snapshots illustrate the catalytic cycle of β-galactocerebrosidase, the defective enzyme in Krabbe disease.New therapeutic approaches for Krabbe disease: The potential of pharmacological chaperones Molecular pharming's foot in the FDA's door: Protalix's trailblazing story Identification of pharmacological chaperones for Gaucher disease and characterization of their effects on beta-glucocerebrosidase by hydrogen/deuterium exchange mass spectrometry.Comparative therapeutic effects of velaglucerase alfa and imiglucerase in a Gaucher disease mouse model.The development and use of small molecule inhibitors of glycosphingolipid metabolism for lysosomal storage diseases.ELIGLUSTAT TARTRATE: Glucosylceramide Synthase Inhibitor Treatment of Type 1 Gaucher Disease.X-ray and biochemical analysis of N370S mutant human acid β-glucosidase.Pharmacological chaperone therapy for lysosomal storage diseases.Azasugar inhibitors as pharmacological chaperones for Krabbe disease In vitro studies of novel PRKAR1A mutants that extend the predicted RIα protein sequence into the 3'-untranslated open reading frame: proteasomal degradation leads to RIα haploinsufficiency and Carney complex Covalent inhibitors of glycosidases and their applications in biochemistry and biology.Glycosylation and functionality of recombinant β-glucocerebrosidase from various production systems.Glycosphingolipids--nature, function, and pharmacological modulation.A Guided Tour of the Structural Biology of Gaucher Disease: Acid-β-Glucosidase and Saposin C.Glycomimetic-based pharmacological chaperones for lysosomal storage disorders: lessons from Gaucher, GM1-gangliosidosis and Fabry diseases.Design, synthesis, and biological evaluation of N-alkylated deoxynojirimycin (DNJ) derivatives for the treatment of dengue virus infection.Stabilization of Glucocerebrosidase by Active Site Occupancy.Participation of asparagine 370 and glutamine 235 in the catalysis by acid beta-glucosidase: the enzyme deficient in Gaucher disease.β-Glucuronidase from Lactobacillus brevis useful for baicalin hydrolysis belongs to glycoside hydrolase family 30.New insights into the pharmacological chaperone activity of c2-substituted glucoimidazoles for the treatment of Gaucher disease.Ethylenedioxy-PIP2 oxalate reduces ganglioside storage in juvenile Sandhoff disease mice.Accessing 2-substituted piperidine iminosugars by organometallic addition/intramolecular reductive amination: aldehyde vs. nitrone route.Synthetic deoxynojirimycin derivatives bearing a thiolated, fluorinated or unsaturated N-alkyl chain: identification of potent α-glucosidase and trehalase inhibitors as well as F508del-CFTR correctors.Mass spectrometric study of gas-phase ions of acid β-glucosidase (Cerezyme) and iminosugar pharmacological chaperones.Tuning protein folding in lysosomal storage diseases: the chemistry behind pharmacological chaperones.

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P2860

Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease

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Crystal structures of complexe ...... rone action in Gaucher disease

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Crystal structures of complexe ...... rone action in Gaucher disease

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Crystal structures of complexe ...... rone action in Gaucher disease

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Crystal structures of complexe ...... rone action in Gaucher disease

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Crystal structures of complexe ...... rone action in Gaucher disease

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Crystal structures of complexe ...... rone action in Gaucher disease

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Crystal structures of complexe ...... rone action in Gaucher disease

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Crystal structures of complexe ...... rone action in Gaucher disease

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Harry M Greenblatt

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Yoseph Shaaltiel

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P2860

Processing of X-ray diffraction data collected in oscillation mode

X-ray structure of human acid-β-glucosidase, the defective enzyme in Gaucher disease

First crystallographic structure of a xylanase from glycoside hydrolase family 5: implications for catalysis

Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease

Structural and mechanistic analyses of endo-glycoceramidase II, a membrane-associated family 5 glycosidase in the Apo and GM3 ganglioside-bound forms

Production of glucocerebrosidase with terminal mannose glycans for enzyme replacement therapy of Gaucher's disease using a plant cell system

Coot: model-building tools for molecular graphics

Refinement of macromolecular structures by the maximum-likelihood method

LIGPLOT: a program to generate schematic diagrams of protein-ligand interactions

Structures and mechanisms of glycosyl hydrolases

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10.1074/JBC.M705005200

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282

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molecular chaperones

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