Structural comparison of differently glycosylated forms of acid-β-glucosidase, the defective enzyme in Gaucher disease
about
Molecular basis of reduced glucosylceramidase activity in the most common Gaucher disease mutant, N370SProduction of glucocerebrosidase with terminal mannose glycans for enzyme replacement therapy of Gaucher's disease using a plant cell systemCrystal 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 StabilityCharacterization of gene-activated human acid- -glucosidase: Crystal structure, glycan composition, and internalization into macrophagesIdentification of pharmacological chaperones for Gaucher disease and characterization of their effects on beta-glucocerebrosidase by hydrogen/deuterium exchange mass spectrometry.Detection of ligand binding hot spots on protein surfaces via fragment-based methods: application to DJ-1 and glucocerebrosidase.Proteome-wide analysis of single-nucleotide variations in the N-glycosylation sequon of human genesX-ray and biochemical analysis of N370S mutant human acid β-glucosidase.Structural features of membrane-bound glucocerebrosidase and α-synuclein probed by neutron reflectometry and fluorescence spectroscopy.Pharmacological chaperone therapy for lysosomal storage diseases.Molecular imaging of membrane interfaces reveals mode of beta-glucosidase activation by saposin C.Characterization of the complex formed by β-glucocerebrosidase and the lysosomal integral membrane protein type-2.Gaucher disease: mutation and polymorphism spectrum in the glucocerebrosidase gene (GBA).Covalent inhibitors of glycosidases and their applications in biochemistry and biology.A Guided Tour of the Structural Biology of Gaucher Disease: Acid-β-Glucosidase and Saposin C.Genetic convergence of Parkinson's disease and lysosomal storage disorders.Consolidation of glycosyl hydrolase family 30: a dual domain 4/7 hydrolase family consisting of two structurally distinct groups.Participation of asparagine 370 and glutamine 235 in the catalysis by acid beta-glucosidase: the enzyme deficient in Gaucher disease.Endosperm-specific expression of human acid beta-glucosidase in a waxy rice
P2860
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P2860
Structural comparison of differently glycosylated forms of acid-β-glucosidase, the defective enzyme in Gaucher disease
description
im November 2006 veröffentlichter wissenschaftlicher Artikel
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scientific article published on 23 November 2006
@en
wetenschappelijk artikel
@nl
наукова стаття, опублікована в листопаді 2006
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name
Structural comparison of diffe ...... tive enzyme in Gaucher disease
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Structural comparison of diffe ...... tive enzyme in Gaucher disease
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type
label
Structural comparison of diffe ...... tive enzyme in Gaucher disease
@en
Structural comparison of diffe ...... tive enzyme in Gaucher disease
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prefLabel
Structural comparison of diffe ...... tive enzyme in Gaucher disease
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Structural comparison of diffe ...... tive enzyme in Gaucher disease
@nl
P50
P1476
Structural comparison of diffe ...... tive enzyme in Gaucher disease
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P2093
Boris Brumshtein
Israel Silman
P304
P356
10.1107/S0907444906038303
P577
2006-11-23T00:00:00Z