(Q36951289)

17 August 2017

1 reference

title

Pharmacologic chaperoning as a strategy to treat Gaucher disease (English)

1 reference

17 August 2017

0 references

1 reference

3

Jeffery W Kelly

1 reference

17 August 2017

Zhanqian Yu

1

1 reference

PubMed ID

17894779

17 August 2017

Anu R Sawkar

series ordinal

2

1 reference

PubMed ID

17894779

17 August 2017

language of work or name

English

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publication date

1 October 2007

1 reference

17 August 2017

1 reference

17 August 2017

274

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17 August 2017

19

1 reference

17 August 2017

4944-4950

1 reference

The cell biology of lysosomal storage disorders

17 August 2017

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

The pathogenesis of glycosphingolipid storage disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

Gaucher disease: Perspectives on a prototype lysosomal disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

Human Gene Mutation Database (HGMD): 2003 update.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

Effectiveness of enzyme replacement therapy in 1028 patients with type 1 Gaucher disease after 2 to 5 years of treatment: a report from the Gaucher Registry

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

The role of the iminosugar N-butyldeoxynojirimycin (miglustat) in the management of type I (non-neuronopathic) Gaucher disease: a position statement

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

Correcting temperature-sensitive protein folding defects

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

Characterization of a mutant alpha-galactosidase gene product for the late-onset cardiac form of Fabry disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

Improvement in cardiac function in the cardiac variant of Fabry's disease with galactose-infusion therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

Active-site-specific chaperone therapy for Fabry disease. Yin and Yang of enzyme inhibitors

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

Alpha-1-C-octyl-1-deoxynojirimycin as a pharmacological chaperone for Gaucher disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2007.06042.X

10.1111/J.1742-4658.2007.06042.X

21 January 2018

Identifiers

DOI

1 reference

17 August 2017

1 reference

PubMed ID

17894779