Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models. - Wikidata - awgldk - TriplyDB

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.

http://www.wikidata.org/entity/Q35066495

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Model systems of protein-misfolding diseases reveal chaperone modifiers of proteotoxicity Inside out: the role of nucleocytoplasmic transport in ALS and FTLD Dysregulated axonal RNA translation in amyotrophic lateral sclerosis RNA-binding proteins, neural development and the addictions RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress response TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Review: Modulating the unfolded protein response to prevent neurodegeneration and enhance memory Engineering enhanced protein disaggregases for neurodegenerative disease Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?Drosophila as an In Vivo Model for Human Neurodegenerative Disease Hnrnph1 Is A Quantitative Trait Gene for Methamphetamine Sensitivity Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell death Mitofusin-mediated ER stress triggers neurodegeneration in pink1/parkin models of Parkinson's disease Repurposed drugs targeting eIF2α-P-mediated translational repression prevent neurodegeneration in mice.Modelling amyotrophic lateral sclerosis: progress and possibilities Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis.Poly(A)-binding proteins are required for diverse biological processes in metazoans.Poly-A binding protein-1 localization to a subset of TDP-43 inclusions in amyotrophic lateral sclerosis occurs more frequently in patients harboring an expansion in C9orf72 Molecular network analysis suggests a logical hypothesis for the pathological role of c9orf72 in amyotrophic lateral sclerosis/frontotemporal dementia.Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins.Functions of FUS/TLS from DNA repair to stress response: implications for ALS.Targeted exon capture and sequencing in sporadic amyotrophic lateral sclerosis.miRNAs at the interface of cellular stress and disease Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity.Interaction of tau with the RNA-Binding Protein TIA1 Regulates tau Pathophysiology and Toxicity.Genome-Scale Networks Link Neurodegenerative Disease Genes to α-Synuclein through Specific Molecular Pathways.Dysregulation of RNA Binding Protein Aggregation in Neurodegenerative Disorders.Opposing roles of p38 and JNK in a Drosophila model of TDP-43 proteinopathy reveal oxidative stress and innate immunity as pathogenic components of neurodegeneration.Clioquinol promotes the degradation of metal-dependent amyloid-β (Aβ) oligomers to restore endocytosis and ameliorate Aβ toxicity.Expression of FSHD-related DUX4-FL alters proteostasis and induces TDP-43 aggregation Somatostatin, neuronal vulnerability and behavioral emotionality.A fruitful endeavor: modeling ALS in the fruit fly Isolating potentiated Hsp104 variants using yeast proteinopathy models Linking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis.Pharmacological dimerization and activation of the exchange factor eIF2B antagonizes the integrated stress response.A Structural and Functional Comparison Between Infectious and Non-Infectious Autocatalytic Recombinant PrP Conformers.Guanabenz Treatment Accelerates Disease in a Mutant SOD1 Mouse Model of ALS.Gene expression and functional annotation of human choroid plexus epithelium failure in Alzheimer's disease.Altered mRNP granule dynamics in FTLD pathogenesis.PABPN1 suppresses TDP-43 toxicity in ALS disease models.

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Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.

http://www.wikidata.org/entity/Q35066495

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Therapeutic modulation of eIF2 ...... eral sclerosis disease models.

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Aaron D Gitler

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Alya R Raphael

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Eva S LaDow

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Hyung-Jun Kim

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Leeanne McGurk

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Nancy M Bonini

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Ross A Weber

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Steven Finkbeiner

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Virginia M-Y Lee

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P2860

Ataxin-2 and its Drosophila homolog, ATX2, physically assemble with polyribosomes

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules

Characterization of mammalian eIF2A and identification of the yeast homolog

Sustained translational repression by eIF2α-P mediates prion neurodegeneration

Altered ribostasis: RNA-protein granules in degenerative disorders

Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

Alpha-synuclein blocks ER-Golgi traffic and Rab1 rescues neuron loss in Parkinson's models

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis

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