(Q37038867)

17 August 2017

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title

A counterintuitive approach to treat enzyme deficiencies: use of enzyme inhibitors for restoring mutant enzyme activity (English)

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17 August 2017

Jian-Qiang Fan

1

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17 August 2017

publication date

1 January 2008

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17 August 2017

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17 August 2017

volume

389

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17 August 2017

issue

1

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17 August 2017

page(s)

1-11

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Novel alpha-L-fucosidase inhibitors from the bark of Angylocalyx pynaertii (Leguminosae).

17 August 2017

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METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE.

21 January 2018

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Specific inhibition of human beta-D-glucuronidase and alpha-L-iduronidase by a trihydroxy pipecolic acid of plant origin.

21 January 2018

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Therapeutic approaches to protein-misfolding diseases

21 January 2018

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Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.

21 January 2018

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

21 January 2018

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Quality control in the endoplasmic reticulum

21 January 2018

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Setting the standards: quality control in the secretory pathway

21 January 2018

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Gene therapy for lysosomal storage diseases: the lessons and promise of animal models

21 January 2018

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Treatment of lysosomal storage disorders: cell therapy and gene therapy

21 January 2018

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

21 January 2018

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

21 January 2018

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Molecular chaperones in the cytosol: from nascent chain to folded protein

21 January 2018

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Roles of N-linked glycans in the endoplasmic reticulum

21 January 2018

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Reversal of clinical features of Hurler's disease and biochemical improvement after treatment by bone-marrow transplantation

21 January 2018

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Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin

21 January 2018

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Bone marrow transplantation as effective treatment of central nervous system disease in globoid cell leukodystrophy, metachromatic leukodystrophy, adrenoleukodystrophy, mannosidosis, fucosidosis, aspartylglucosaminuria, Hurler, Maroteaux-Lamy, and S

21 January 2018

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Quantitative correlation between the residual activity of beta-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease

21 January 2018

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Pyrimethamine as a potential pharmacological chaperone for late-onset forms of GM2 gangliosidosis.

21 January 2018

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Chemical chaperone therapy for brain pathology in G(M1)-gangliosidosis

21 January 2018

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The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation

21 January 2018

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Prevalence of lysosomal storage disorders

21 January 2018

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Chemical modification of the beta-glucocerebrosidase inhibitor N-octyl-beta-valienamine: synthesis and biological evaluation of 4-epimeric and 4-O-(beta-D-galactopyranosyl) derivatives

21 January 2018

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Pharmacological enhancement of mutated alpha-glucosidase activity in fibroblasts from patients with Pompe disease

21 January 2018

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Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells.

21 January 2018

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Thirty years of Batten disease research: present status and future goals

21 January 2018

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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/10.1515%2FBC.2008.009

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

21 January 2018

1 reference

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New prospects for the treatment of lysosomal storage diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2008.009

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2008.009

Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2008.009

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/10.1515%2FBC.2008.009

Pharmacological enhancement of beta-hexosaminidase activity in fibroblasts from adult Tay-Sachs and Sandhoff Patients.

21 January 2018

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https://api.crossref.org/works/10.1515%2FBC.2008.009

High-throughput screening for human lysosomal beta-N-Acetyl hexosaminidase inhibitors acting as pharmacological chaperones

21 January 2018

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https://api.crossref.org/works/10.1515%2FBC.2008.009

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

21 January 2018

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https://api.crossref.org/works/10.1515%2FBC.2008.009

A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorder

21 January 2018

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https://api.crossref.org/works/10.1515%2FBC.2008.009

Not such a dismal science: the economics of protein synthesis, folding, degradation and antigen processing

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2008.009

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2008.009

Nagstatin, a new inhibitor of N-acetyl-beta-D-glucosaminidase, produced by Streptomyces amakusaensis MG846-fF3. Taxonomy, production, isolation, physico-chemical properties and biological activities

21 January 2018

1 reference

12 April 2024

https://opencitations.net/index/api/v1/citations/10.7164/ANTIBIOTICS.45.1404

Mannostatins A and B: new inhibitors of alpha-D-mannosidase, produced by Streptoverticillium verticillus var. quintum ME3-AG3: taxonomy, production, isolation, physico-chemical properties and biological activities.

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12 April 2024

https://opencitations.net/index/api/v1/citations/10.7164/ANTIBIOTICS.42.883

Purification and characterization of a sialidase inhibitor, siastatin, produced by Streptomyces

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12 April 2024

https://opencitations.net/index/api/v1/citations/10.7164/ANTIBIOTICS.27.963

Identifiers

DOI

10.1515/BC.2008.009

1 reference

17 August 2017

1 reference