Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease - Test2 - elhamkhani - TriplyDB

Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease

Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease

http://www.wikidata.org/entity/Q24602248

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Human neutrophils secrete bioactive paucimannosidic proteins from azurophilic granules into pathogen-infected sputum Therapeutic potential of intracerebroventricular replacement of modified human β-hexosaminidase B for GM2 gangliosidosis An open-label Phase I/II clinical trial of pyrimethamine for the treatment of patients affected with chronic GM2 gangliosidosis (Tay-Sachs or Sandhoff variants)Crystallographic structure of human beta-hexosaminidase A: interpretation of Tay-Sachs mutations and loss of GM2 ganglioside hydrolysis Small molecule inhibitors of a glycoside hydrolase attenuate inducible AmpC-mediated beta-lactam resistance GlcNAcstatins are nanomolar inhibitors of human O -GlcNAcase inducing cellular hyper- O -GlcNAcylation Insight into a strategy for attenuating AmpC-mediated β-lactam resistance: Structural basis for selective inhibition of the glycoside hydrolase NagZ The structure of a family GH25 lysozyme fromAspergillus fumigatus Cell-Penetrant, Nanomolar O-GlcNAcase Inhibitors Selective against Lysosomal Hexosaminidases Structural Determinants of an Insect -N-Acetyl-D-hexosaminidase Specialized as a Chitinolytic Enzyme Active-pocket size differentiating insectile from bacterial chitinolytic β-N-acetyl-D-hexosaminidases Crystal Structures of a Glycoside Hydrolase Family 20 Lacto-N-biosidase from Bifidobacterium bifidum Gaining insight into the inhibition of glycoside hydrolase family 20 exo-β-N-acetylhexosaminidases using a structural approach Metabolism of Vertebrate Amino Sugars with N-Glycolyl Groups: INTRACELLULAR -O-LINKED N-GLYCOLYLGLUCOSAMINE (GlcNGc), UDP-GlcNGc, AND THE BIOCHEMICAL AND STRUCTURAL RATIONALE FOR THE SUBSTRATE TOLERANCE OF -O-LINKED -N-ACETYLGLUCOSAMINIDASE Characterization of the biosynthesis, processing and kinetic mechanism of action of the enzyme deficient in mucopolysaccharidosis IIIC Structure and mechanism of a bacterial beta-glucosaminidase having O-GlcNAcase activity Structural-Functional Analysis Reveals a Specific Domain Organization in Family GH20 Hexosaminidases Probing the Catalytic Mechanism of Vibrio harveyi GH20 β-N-Acetylglucosaminidase by Chemical Rescue A crystal structure-guided rational design switching non-carbohydrate inhibitors' specificity between two β-GlcNAcase homologs Tay-Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum-associated degradation Application of protein engineering to enhance crystallizability and improve crystal properties.Structure of the dimeric N-glycosylated form of fungal beta-N-acetylhexosaminidase revealed by computer modeling, vibrational spectroscopy, and biochemical studies.Pyrimethamine as a potential pharmacological chaperone for late-onset forms of GM2 gangliosidosis.Photoaffinity labelling of the human GM2-activator protein. Mechanistic insight into ganglioside GM2 degradation.The natural history of juvenile or subacute GM2 gangliosidosis: 21 new cases and literature review of 134 previously reported.Molecular and biochemical characterization of a novel β-N-acetyl-D-hexosaminidase with broad substrate-spectrum from the Aisan corn borer, Ostrinia furnacalis.Purification, characterization, and cloning of a Spodoptera frugiperda Sf9 beta-N-acetylhexosaminidase that hydrolyzes terminal N-acetylglucosamine on the N-glycan core.High-throughput screening for human lysosomal beta-N-Acetyl hexosaminidase inhibitors acting as pharmacological chaperones.Lending a helping hand, screening chemical libraries for compounds that enhance beta-hexosaminidase A activity in GM2 gangliosidosis cells Phylogenetic analyses suggest multiple changes of substrate specificity within the glycosyl hydrolase 20 family.A sensitive fluorescence-based assay for monitoring GM2 ganglioside hydrolysis in live patient cells and their lysates Pharmacological enhancement of beta-hexosaminidase activity in fibroblasts from adult Tay-Sachs and Sandhoff Patients.Crystal structure of β-hexosaminidase B in complex with pyrimethamine, a potential pharmacological chaperone Sequence and copy number analyses of HEXB gene in patients affected by Sandhoff disease: functional characterization of 9 novel sequence variants.Comparative Transcriptomic Exploration Reveals Unique Molecular Adaptations of Neuropathogenic Trichobilharzia to Invade and Parasitize Its Avian Definitive Host.In cellulo examination of a beta-alpha hybrid construct of beta-hexosaminidase A subunits, reported to interact with the GM2 activator protein and hydrolyze GM2 ganglioside Role of active-site residues of dispersin B, a biofilm-releasing beta-hexosaminidase from a periodontal pathogen, in substrate hydrolysis.Crystallization and diffraction analysis of β-N-acetylhexosaminidase from Aspergillus oryzae.Incidence and carrier frequency of Sandhoff disease in Saskatchewan determined using a novel substrate with detection by tandem mass spectrometry and molecular genetic analysis.Computational study of β-N-acetylhexosaminidase from Talaromyces flavus, a glycosidase with high substrate flexibility

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Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease

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2003 nî lūn-bûn

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2003 թուականի Ապրիլին հրատարակուած գիտական յօդուած

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2003年の論文

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Journal of Molecular Biology

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Crystal structure of human bet ...... Sandhoff and Tay-Sachs disease

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Brian L Mark

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Dalian Zhao

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Don J Mahuran

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Maia M Cherney

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Michael N G James

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Spencer Knapp

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P2860

New families in the classification of glycosyl hydrolases based on amino acid sequence similarities

Serratia marcescens chitobiase is a retaining glycosidase utilizing substrate acetamido group participation

Several cooperating binding sites mediate the interaction of a lysosomal enzyme with phosphotransferase

Updating the sequence-based classification of glycosyl hydrolases

Molecular surface recognition: determination of geometric fit between proteins and their ligands by correlation techniques

Characterization of the Glu and Asp residues in the active site of human beta-hexosaminidase B

Complete localization of disulfide bonds in GM2 activator protein

GM2-gangliosidosis B1 variant: analysis of beta-hexosaminidase alpha gene mutations in 11 patients from a defined region in Portugal

Improved methods for building protein models in electron density maps and the location of errors in these models

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crystal structure

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