Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease
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Molecular basis of reduced glucosylceramidase activity in the most common Gaucher disease mutant, N370SStructural and mechanistic insight into the basis of mucopolysaccharidosis IIIBPharmacological chaperoning: a primer on mechanism and pharmacologyChemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosisCrystal 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 diseaseAcid beta-glucosidase: insights from structural analysis and relevance to Gaucher disease therapyEffects of pH and Iminosugar Pharmacological Chaperones on Lysosomal Glycosidase Structure and StabilityCrystal Structure of the Salmonella enterica Serovar Typhimurium Virulence Factor SrfJ, a Glycoside Hydrolase Family EnzymeCyclodextrin-mediated crystallization of acid β-glucosidase in complex with amphiphilic bicyclic nojirimycin analoguesInsights into Krabbe disease from structures of galactocerebrosidaseBinding of 3,4,5,6-Tetrahydroxyazepanes to the Acid-β-glucosidase Active Site: Implications for Pharmacological Chaperone Design for Gaucher DiseaseStructural snapshots illustrate the catalytic cycle of β-galactocerebrosidase, the defective enzyme in Krabbe disease.Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasisIn vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse modelsScreening, production, optimization and characterization of β-glucosidase using microbes from shellfish wasteQuantitative assessment of protein structural models by comparison of H/D exchange MS data with exchange behavior accurately predicted by DXCOREX.Small-molecule structure correctors target abnormal protein structure and function: structure corrector rescue of apolipoprotein E4-associated neuropathologyStrategies for stabilizing superoxide dismutase (SOD1), the protein destabilized in the most common form of familial amyotrophic lateral sclerosis.Three classes of glucocerebrosidase inhibitors identified by quantitative high-throughput screening are chaperone leads for Gaucher diseaseIdentification of pharmacological chaperones for Gaucher disease and characterization of their effects on beta-glucocerebrosidase by hydrogen/deuterium exchange mass spectrometry.Optimization and validation of two miniaturized glucocerebrosidase enzyme assays for high throughput screening.Detection of ligand binding hot spots on protein surfaces via fragment-based methods: application to DJ-1 and glucocerebrosidase.Identification and characterization of ambroxol as an enzyme enhancement agent for Gaucher diseaseComparative therapeutic effects of velaglucerase alfa and imiglucerase in a Gaucher disease mouse model.Insights into Hunter syndrome from the structure of iduronate-2-sulfatasePharmacological chaperones stabilize retromer to limit APP processing.The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase.Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse.Potential pharmacological chaperones targeting cancer-associated MCL-1 and Parkinson disease-associated α-synuclein.Engineered disulfide bonds restore chaperone-like function of DJ-1 mutants linked to familial Parkinson's disease.Pharmacological chaperones in the age of proteomic pathology.Discovery, structure-activity relationship, and biological evaluation of noninhibitory small molecule chaperones of glucocerebrosidaseX-ray and biochemical analysis of N370S mutant human acid β-glucosidase.Identification and characterization of pharmacological chaperones to correct enzyme deficiencies in lysosomal storage disorders.Evaluation of quinazoline analogues as glucocerebrosidase inhibitors with chaperone activityAlpha-synuclein interacts with Glucocerebrosidase providing a molecular link between Parkinson and Gaucher diseasesSneak peak at galactocerebrosidase, Krabbe disease's lysosomal hydrolaseA chaperone enhances blood α-glucosidase activity in Pompe disease patients treated with enzyme replacement therapy.Histone deacetylase inhibitors prevent the degradation and restore the activity of glucocerebrosidase in Gaucher diseaseAzasugar inhibitors as pharmacological chaperones for Krabbe disease
P2860
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P2860
Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease
description
2007 nî lūn-bûn
@nan
2007 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2007年の論文
@ja
2007年学术文章
@wuu
2007年学术文章
@zh-cn
2007年学术文章
@zh-hans
2007年学术文章
@zh-my
2007年学术文章
@zh-sg
2007年學術文章
@yue
name
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@ast
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@en
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@nl
type
label
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@ast
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@en
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@nl
prefLabel
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@ast
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@en
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@nl
P2093
P921
P3181
P356
P1476
Structure of acid beta-glucosi ...... s insight into Gaucher disease
@en
P2093
Allan C Powe
Brandon A Wustman
Corey W Pine
Michael G Schlossmacher
Pedro Huertas
Raquel L Lieberman
Richie Khanna
P2888
P3181
P356
10.1038/NCHEMBIO850
P577
2007-02-01T00:00:00Z
P6179
1047184309