Ultrasensitive in situ visualization of active glucocerebrosidase molecules (Q82463943)

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16 January 2020

title

Ultrasensitive in situ visualization of active glucocerebrosidase molecules (English)

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2

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Gertjan Kramer

9

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Rolf G Boot

14

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Johannes Aerts

17

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Wilma E Donker-Koopman

6

object named as

Wilma E Donker-Koopman

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16

Herman S Overkleeft

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12

Carla E M Hollak

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13

Roelof Ottenhoff

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5

Anneke Strijland

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author name string

Martin D Witte

1

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Jan Aten

3

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Kah-Yee Li

4

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Adrianus M C H van den Nieuwendijk

7

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Boris Bleijlevens

8

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Bogdan I Florea

10

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Berend Hooibrink

11

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Gijsbert A van der Marel

15

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publication date

31 October 2010

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16 January 2020

published in

Nature Chemical Biology

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volume

6

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issue

12

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page(s)

907-913

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

16 January 2020

cites work

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12 December 2020

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

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12 December 2020

Clinical phenotype of Gaucher disease in relation to properties of mutant glucocerebrosidase in cultured fibroblasts

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12 December 2020

Imaging of enzyme replacement therapy using PET

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12 December 2020

Chemical and biological approaches synergize to ameliorate protein-folding diseases.

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12 December 2020

Characterization of human glucocerebrosidase from different mutant alleles

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12 December 2020

Demonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher's disease

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12 December 2020

A procedure for the rapid purification in high yield of human glucocerebrosidase using immunoaffinity chromatography with monoclonal antibodies

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12 December 2020

N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.

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12 December 2020

Isofagomine induced stabilization of glucocerebrosidase

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12 December 2020

Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention.

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12 December 2020

Role of pH in determining the cell-type-specific residual activity of glucocerebrosidase in type 1 Gaucher disease

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12 December 2020

Glucocerebrosidase, a lysosomal enzyme that does not undergo oligosaccharide phosphorylation

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12 December 2020

Replacement therapy for inherited enzyme deficiency--macrophage-targeted glucocerebrosidase for Gaucher's disease

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12 December 2020

Glucocerebrosidase genotype of Gaucher patients in The Netherlands: limitations in prognostic value

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12 December 2020

Twin pairs showing discordance of phenotype in adult Gaucher's disease

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12 December 2020

Participation of asparagine 370 and glutamine 235 in the catalysis by acid beta-glucosidase: the enzyme deficient in Gaucher disease

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12 December 2020

Chemical chaperones increase the cellular activity of N370S beta -glucosidase: a therapeutic strategy for Gaucher disease

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12 December 2020

Gaucher disease-associated glucocerebrosidases show mutation-dependent chemical chaperoning profiles

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12 December 2020

Phenotype, diagnosis, and treatment of Gaucher's disease

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12 December 2020

A short synthesis of ( )-cyclophellitol

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12 December 2020

N-butyldeoxygalactonojirimycin: a more selective inhibitor of glycosphingolipid biosynthesis than N-butyldeoxynojirimycin, in vitro and in vivo

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12 December 2020

The spectrum of parkinsonian manifestations associated with glucocerebrosidase mutations

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12 December 2020

Covalent inhibitors of glycosidases and their applications in biochemistry and biology

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12 December 2020

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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12 December 2020

Biosynthesis and maturation of glucocerebrosidase in Gaucher fibroblasts

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12 December 2020

Isofagomine increases lysosomal delivery of exogenous glucocerebrosidase

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12 December 2020

Enzyme therapy in type 1 Gaucher disease: comparative efficacy of mannose-terminated glucocerebrosidase from natural and recombinant sources

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12 December 2020

ER retention and degradation as the molecular basis underlying Gaucher disease heterogeneity

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12 December 2020

Hydrophilic iminosugar active-site-specific chaperones increase residual glucocerebrosidase activity in fibroblasts from Gaucher patients

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12 December 2020

Structural basis for cyclophellitol inhibition of a beta-glucosidase

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12 December 2020

Generation of specific deoxynojirimycin-type inhibitors of the non-lysosomal glucosylceramidase

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12 December 2020

Differential mechanism-based labeling and unequivocal activity assignment of the two active sites of intestinal lactase/phlorizin hydrolase.

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12 December 2020

Iminosugar glycosidase inhibitors: structural and thermodynamic dissection of the binding of isofagomine and 1-deoxynojirimycin to beta-glucosidases

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12 December 2020

Fluorescent flow cytometric assay: a new diagnostic tool for measuring beta-glucocerebrosidase activity in Gaucher disease

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12 December 2020

Acid beta-glucosidase: insights from structural analysis and relevance to Gaucher disease therapy

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12 December 2020

Accumulation of tissue glucosylsphingosine in Gaucher-like mouse induced by the glucosylceramidase inhibitor cyclophellitol

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12 December 2020

Substrate reduction therapy of glycosphingolipid storage disorders.

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12 December 2020

Identifiers

DOI

10.1038/NCHEMBIO.466

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16 January 2020

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