X-ray and biochemical analysis of N370S mutant human acid β-glucosidase. (Q34452544)

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14 January 2020

title

X-ray and biochemical analysis of N370S mutant human acid β-glucosidase (English)

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4

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14 January 2020

author name string

Ronnie R Wei

1

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14 January 2020

Heather Hughes

2

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14 January 2020

Susan Boucher

3

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14 January 2020

Nicholas Guziewicz

5

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14 January 2020

Scott M Van Patten

6

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14 January 2020

Huawei Qiu

7

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14 January 2020

Clark Qun Pan

8

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14 January 2020

Tim Edmunds

9

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14 January 2020

language of work or name

English

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publication date

27 October 2010

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Journal of Biological Chemistry

14 January 2020

published in

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volume

286

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14 January 2020

page(s)

299-308

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issue

1

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Characterization of gene-activated human acid- -glucosidase: Crystal structure, glycan composition, and internalization into macrophages

14 January 2020

cites work

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6 July 2018

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

6 July 2018

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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

6 July 2018

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Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase

6 July 2018

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

6 July 2018

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

6 July 2018

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Scaling and assessment of data quality

6 July 2018

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Hematologically important mutations: Gaucher disease

6 July 2018

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Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease.

6 July 2018

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

6 July 2018

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6 July 2018

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Refinement of macromolecular structures by the maximum-likelihood method

6 July 2018

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

6 July 2018

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

6 July 2018

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6 July 2018

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Analysis of human acid beta-glucosidase by site-directed mutagenesis and heterologous expression.

6 July 2018

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

6 July 2018

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Gaucher's disease: deficiency of 'acid' -glucosidase and reconstitution of enzyme activity in vitro

6 July 2018

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Characterization of the activation of rat liver beta-glucosidase by sialosylgangliotetraosylceramide

6 July 2018

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6 July 2018

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6 July 2018

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6 July 2018

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6 July 2018

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6 July 2018

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6 July 2018

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

21 January 2018

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Chemical chaperones and permissive temperatures alter localization of Gaucher disease associated glucocerebrosidase variants

21 January 2018

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Structural comparison of differently glycosylated forms of acid-β-glucosidase, the defective enzyme in Gaucher disease

21 January 2018

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Use of direct fluorescence labeling and confocal microscopy to determine the biodistribution of two protein therapeutics, Cerezyme and Ceredase

21 January 2018

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12 December 2020

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12 December 2020

Effects of detergents and choline-containing phospholipids on human spleen glucocerebrosidase

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Biochemical properties of β-glucosidase in leukocytes from patients and obligated heterozygotes for Gaucher disease carriers

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Identifiers

DOI

10.1074/JBC.M110.150433

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14 January 2020

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14 January 2020

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