(Q24630192)

1

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Wensi Song

2

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Giovanna Brancati

3

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Laura Segatori

Journal of Biological Chemistry

4

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language of work or name

English

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publication date

16 December 2011

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published in

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volume

286

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issue

50

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page(s)

43454-64

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cites work

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Endoplasmic reticulum (ER) mannosidase I is compartmentalized and required for N-glycan trimming to Man5-6GlcNAc2 in glycoprotein ER-associated degradation

7 April 2017

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Roles of CHOP/GADD153 in endoplasmic reticulum stress

7 April 2017

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Signal integration in the endoplasmic reticulum unfolded protein response

7 April 2017

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Decreased glucocerebrosidase activity in Gaucher disease parallels quantitative enzyme loss due to abnormal interaction with TCP1 and c-Cbl.

7 April 2017

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Molecular chaperones in the yeast endoplasmic reticulum maintain the solubility of proteins for retrotranslocation and degradation

7 April 2017

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The ERAD inhibitor Eeyarestatin I is a bifunctional compound with a membrane-binding domain and a p97/VCP inhibitory group

7 April 2017

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Dissection of the dislocation pathway for type I membrane proteins with a new small molecule inhibitor, eeyarestatin

7 April 2017

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The mammalian unfolded protein response

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Ca2+ homeostasis modulation enhances the amenability of L444P glucosylcerebrosidase to proteostasis regulation in patient-derived fibroblasts

7 April 2017

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Current enzyme replacement therapy for the treatment of lysosomal storage diseases.

27 September 2017

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

27 September 2017

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Gaucher disease: mutation and polymorphism spectrum in the glucocerebrosidase gene (GBA).

27 September 2017

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The recognition and retrotranslocation of misfolded proteins from the endoplasmic reticulum

27 September 2017

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Treatment of lysosomal storage disorders : progress with enzyme replacement therapy

27 September 2017

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

27 September 2017

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Therapy for Gaucher disease: don't stop thinking about tomorrow

27 September 2017

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A window of opportunity: timing protein degradation by trimming of sugars and ubiquitins

27 September 2017

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

27 September 2017

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The cell biology of lysosomal storage disorders

27 September 2017

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

27 September 2017

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Enhancement of endoplasmic reticulum (ER) degradation of misfolded Null Hong Kong alpha1-antitrypsin by human ER mannosidase I.

27 September 2017

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Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis

27 September 2017

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Gaucher disease: gene frequencies and genotype/phenotype correlations

27 September 2017

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Assembly of ER-associated protein degradation in vitro: dependence on cytosol, calnexin, and ATP.

27 September 2017

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Characterization of glycoproteins and their associated oligosaccharides through the use of endoglycosidases

27 September 2017

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

27 September 2017

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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

27 September 2017

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

30 May 2018

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Glycoprotein quality control in the endoplasmic reticulum. Mannose trimming by endoplasmic reticulum mannosidase I times the proteasomal degradation of unassembled immunoglobulin subunits

30 May 2018

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Crossref

https://api.crossref.org/works/10.1074%2FJBC.M111.274332

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

21 January 2018

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https://api.crossref.org/works/10.1074%2FJBC.M111.274332

PoPMuSiC, an algorithm for predicting protein mutant stability changes: application to prion proteins.

21 January 2018

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https://api.crossref.org/works/10.1074%2FJBC.M111.274332

Lacidipine remodels protein folding and Ca 2+ homeostasis in Gaucher's disease fibroblasts: a mechanism to rescue mutant glucocerebrosidase

21 January 2018

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12 December 2020

PoPMuSiC, rationally designing point mutations in protein structures

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12 December 2020

Characterization of glucocerebrosidase in Greek Gaucher disease patients: mutation analysis and biochemical studies

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12 December 2020

Enhanced binding to the molecular chaperone BiP slows thyroglobulin export from the endoplasmic reticulum

1 reference

12 December 2020

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

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12 December 2020