Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease (Q27643401)

GOA release 2020-03-11

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9

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Dagmar Ringe

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Raquel L Lieberman

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object named as

Raquel L Lieberman

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author name string

Brandon A Wustman

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Pedro Huertas

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Allan C Powe

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Corey W Pine

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Richie Khanna

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Michael G Schlossmacher

Therapeutic strategies to ameliorate lysosomal storage disorders--a focus on Gaucher disease

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language of work or name

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February 2007

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published in

Nature Chemical Biology

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volume

3

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issue

2

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page(s)

101-7

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cites work

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7 January 2021

A contradictory treatment for lysosomal storage disorders: inhibitors enhance mutant enzyme activity

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7 January 2021

Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses

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Neuropathology provides clues to the pathophysiology of Gaucher disease

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Glucocerebrosidase mutations are an important risk factor for Lewy body disorders.

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Analyses of variant acid beta-glucosidases: effects of Gaucher disease mutations

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Sustained therapeutic effects of oral miglustat (Zavesca, N-butyldeoxynojirimycin, OGT 918) in type I Gaucher disease.

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An open-label, noncomparative study of miglustat in type I Gaucher disease: efficacy and tolerability over 24 months of treatment

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7 January 2021

Pharmacologic rescue of conformationally-defective proteins: implications for the treatment of human disease

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7 January 2021

Miglustat (NB-DNJ) works as a chaperone for mutated acid beta-glucosidase in cells transfected with several Gaucher disease mutations

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7 January 2021

N-octyl-beta-valienamine up-regulates activity of F213I mutant beta-glucosidase in cultured cells: a potential chemical chaperone therapy for Gaucher disease

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Membrane disruption and cytotoxicity of hydrophobic N-alkylated imino sugars is independent of the inhibition of protein and lipid glycosylation

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Chemical chaperones--a new concept in drug research

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The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms

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7 January 2021

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

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7 January 2021

X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. Implications for Gaucher disease

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7 January 2021

The N370S (Asn370-->Ser) mutation affects the capacity of glucosylceramidase to interact with anionic phospholipid-containing membranes and saposin C

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7 January 2021

Human acid beta-glucosidase: use of inhibitors, alternative substrates and amphiphiles to investigate the properties of the normal and Gaucher disease active sites

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7 January 2021

Mechanism of activation of glucocerebrosidase by CO-β-glucosidase (glucosidase activator protein)

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7 January 2021

Acid beta-glucosidase: intrinsic fluorescence and conformational changes induced by phospholipids and saposin C.

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7 January 2021

Processing of X-ray diffraction data collected in oscillation mode

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The CCP4 suite: programs for protein crystallography

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Coot: model-building tools for molecular graphics

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PRODRG: a tool for high-throughput crystallography of protein-ligand complexes

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Modelling bound ligands in protein crystal structures

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Secondary-structure matching (SSM), a new tool for fast protein structure alignment in three dimensions

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7 January 2021

Intracellular transport of acid beta-glucosidase and lysosome-associated membrane proteins is affected in Gaucher's disease (G202R mutation)

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7 January 2021