Biological and chemical approaches to diseases of proteostasis deficiency
about
Molecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic FibrosisIdentification of human proteins that modify misfolding and proteotoxicity of pathogenic ataxin-1Increased proteasome activity in human embryonic stem cells is regulated by PSMD11A quantitative chaperone interaction network reveals the architecture of cellular protein homeostasis pathwaysSecretion of amyloidogenic gelsolin progressively compromises protein homeostasis leading to the intracellular aggregation of proteinsReduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosisHistone deacetylase inhibitor (HDACi) suberoylanilide hydroxamic acid (SAHA)-mediated correction of α1-antitrypsin deficiencyModel systems of protein-misfolding diseases reveal chaperone modifiers of proteotoxicityOlive Oil and the Hallmarks of AgingMuscle wasting in myotonic dystrophies: a model of premature agingFine-tuning multiprotein complexes using small moleculesProtein folding and quality control in the ERExpanding proteostasis by membrane trafficking networksMolecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosisMolecular chaperones in targeting misfolded proteins for ubiquitin-dependent degradationPathogenic protein seeding in Alzheimer disease and other neurodegenerative disordersComparing protein folding in vitro and in vivo: foldability meets the fitness challengeImpact of the circadian clock on the aging processProtein homeostasis as a therapeutic target for diseases of protein conformationChemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosisThe role of tau in neurodegenerative diseases and its potential as a therapeutic targetThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyProtein quality control in the nucleusNonenzymatic protein acylation as a carbon stress regulated by sirtuin deacylasesThe unstable repeats--three evolving faces of neurological diseaseChaperone machines for protein folding, unfolding and disaggregationHuntington's disease: underlying molecular mechanisms and emerging conceptsA method to rapidly create protein aggregates in living cells.Phosphorylation modulates clearance of alpha-synuclein inclusions in a yeast model of Parkinson's diseaseFunctional diversity and structural disorder in the human ubiquitination pathwayIndividual and collective contributions of chaperoning and degradation to protein homeostasis in E. coli.Amyotrophic lateral sclerosis-linked mutant VAPB inclusions do not interfere with protein degradation pathways or intracellular transport in a cultured cell modelA genetic screening strategy identifies novel regulators of the proteostasis networkA pro-cathepsin L mutant is a luminal substrate for endoplasmic-reticulum-associated degradation in C. elegansInactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in DrosophilaA Substructure Combination Strategy To Create Potent and Selective Transthyretin Kinetic Stabilizers That Prevent Amyloidogenesis and CytotoxicityFlavonol Activation Defines an Unanticipated Ligand-Binding Site in the Kinase-RNase Domain of IRE1Dual Action of ATP Hydrolysis Couples Lid Closure to Substrate Release into the Group II Chaperonin ChamberConformational states and recognition of amyloidogenic peptides of human insulin-degrading enzymeThe Role of Protein Denaturation Energetics and Molecular Chaperones in the Aggregation and Mistargeting of Mutants Causing Primary Hyperoxaluria Type I
P2860
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P2860
Biological and chemical approaches to diseases of proteostasis deficiency
description
2009 nî lūn-bûn
@nan
2009 թուականին հրատարակուած գիտական յօդուած
@hyw
2009 թվականին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
name
Biological and chemical approaches to diseases of proteostasis deficiency
@ast
Biological and chemical approaches to diseases of proteostasis deficiency
@en
type
label
Biological and chemical approaches to diseases of proteostasis deficiency
@ast
Biological and chemical approaches to diseases of proteostasis deficiency
@en
prefLabel
Biological and chemical approaches to diseases of proteostasis deficiency
@ast
Biological and chemical approaches to diseases of proteostasis deficiency
@en
P2093
P3181
P1476
Biological and chemical approaches to diseases of proteostasis deficiency
@en
P2093
P304
P3181
P356
10.1146/ANNUREV.BIOCHEM.052308.114844
P407
P577
2009-01-01T00:00:00Z