Inhibition of endoplasmic reticulum-associated degradation rescues native folding in loss of function protein misfolding diseases - Wikidata - wikimedia - TriplyDB

Inhibition of endoplasmic reticulum-associated degradation rescues native folding in loss of function protein misfolding diseases

Inhibition of endoplasmic reticulum-associated degradation rescues native folding in loss of function protein misfolding diseases

http://www.wikidata.org/entity/Q24630192

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The potential of sarcospan in adhesion complex replacement therapeutics for the treatment of muscular dystrophy Impairment of homeostasis in lysosomal storage disorders Protein homeostasis as a therapeutic target for diseases of protein conformation Type II antithrombin deficiency caused by a large in-frame insertion: structural, functional and pathological relevance A rapid and sensitive method for measuring N-acetylglucosaminidase activity in cultured cells Tay-Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum-associated degradation Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model Hepatitis B virus PreS2-mutant large surface antigen activates store-operated calcium entry and promotes chromosome instability Mitomycin C induces apoptosis in human epidural scar fibroblasts after surgical decompression for spinal cord injury.Alteration of the proteostasis network of plant cells promotes the post-endoplasmic reticulum trafficking of recombinant mutant (L444P) human β-glucocerebrosidase.Remodeling the proteostasis network to rescue glucocerebrosidase variants by inhibiting ER-associated degradation and enhancing ER folding.Combining valosin-containing protein (VCP) inhibition and suberanilohydroxamic acid (SAHA) treatment additively enhances the folding, trafficking, and function of epilepsy-associated γ-aminobutyric acid, type A (GABAA) receptors Combined inhibition of p97 and the proteasome causes lethal disruption of the secretory apparatus in multiple myeloma cells.Pharmacological chaperone therapy for lysosomal storage diseases.Selective Oma1 protease-mediated proteolysis of Cox1 subunit of cytochrome oxidase in assembly mutants.Chemical induction of Hsp70 reduces α-synuclein aggregation in neuroglioma cells.Expanding rare disease drug trials based on shared molecular etiology Impact of Hybrid and Complex N-Glycans on Cell Surface Targeting of the Endogenous Chloride Cotransporter Slc12a2.Inhibitors of the AAA+ chaperone p97 Endoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases.C/EBP homologous protein (CHOP) mediates neuronal apoptosis in rats with spinal cord injury.Recruitment of a cytoplasmic chaperone relay by the A2A adenosine receptor.Partial IFN-γR2 deficiency is due to protein misfolding and can be rescued by inhibitors of glycosylation.Inhibition of endoplasmic reticulum associated degradation reduces endoplasmic reticulum stress and alters lysosomal morphology and distribution.2-Hydroxypropyl-β-cyclodextrin promotes transcription factor EB-mediated activation of autophagy: implications for therapy The interplay between endoplasmic reticulum stress and inflammation.Specificity and regulation of the endoplasmic reticulum-associated degradation machinery.Pharmaceutical Chaperones and Proteostasis Regulators in the Therapy of Lysosomal Storage Disorders: Current Perspective and Future Promises.Molecular Mechanisms of Disease Pathogenesis Differ in Krabbe Disease Variants.Differential regulation of γ-glutamyltransferase and glutamate cysteine ligase expression after mitochondrial uncoupling: γ-glutamyltransferase is regulated in an Nrf2- and NFκB-independent manner.Regulating Secretory Proteostasis through the Unfolded Protein Response: From Function to Therapy.A novel splice variant in EMC1 is associated with cerebellar atrophy, visual impairment, psychomotor retardation with epilepsy.Adapting Secretory Proteostasis and Function Through the Unfolded Protein Response.Proteasome inhibition alleviates SNARE-dependent neurodegeneration.

P2860

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P2860

Inhibition of endoplasmic reticulum-associated degradation rescues native folding in loss of function protein misfolding diseases

http://www.wikidata.org/entity/Q24630192

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2011 nî lūn-bûn

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2011 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած

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Inhibition of endoplasmic reti ...... on protein misfolding diseases

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Inhibition of endoplasmic reti ...... on protein misfolding diseases

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Inhibition of endoplasmic reti ...... on protein misfolding diseases

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Fan Wang

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Giovanna Brancati

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Laura Segatori

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Wensi Song

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P2860

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

Roles of CHOP/GADD153 in endoplasmic reticulum stress

One step at a time: endoplasmic reticulum-associated degradation

Signal integration in the endoplasmic reticulum unfolded protein response

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

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

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

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

The mammalian unfolded protein response

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

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endoplasmic reticulum

protein folding

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