Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice. - Test2 - elhamkhani - TriplyDB

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.

http://www.wikidata.org/entity/Q38288366

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Ataxin-2: From RNA Control to Human Health and Disease Advances in the delivery of RNA therapeutics: from concept to clinical reality.Progression of pathology in PINK1-deficient mouse brain from splicing via ubiquitination, ER stress, and mitophagy changes to neuroinflammation.The Multiple Faces of Spinocerebellar Ataxia type 2.Spinocerebellar Ataxia Type 2: Clinicogenetic Aspects, Mechanistic Insights, and Management Approaches.Neurodegenerative disease: models, mechanisms, and a new hope.Gene suppression approaches to neurodegeneration.Alcohol-abuse drug disulfiram targets cancer via p97 segregase adaptor NPL4.Co-expression Patterns between ATN1 and ATXN2 Coincide with Brain Regions Affected in Huntington's Disease.Endocytosis regulates TDP-43 toxicity and turnover.Motor Neuron Gene Therapy: Lessons from Spinal Muscular Atrophy for Amyotrophic Lateral Sclerosis.Chronic oxidative stress promotes GADD34-mediated phosphorylation of the TAR DNA-binding protein TDP-43, a modification linked to neurodegeneration.Making sense of antisense oligonucleotides: A narrative review.RNA-binding proteins in neurodegeneration: mechanisms in aggregate.Exposure of neonatal mice to bromine impairs their alveolar development and lung function.Polyglutamine spinocerebellar ataxias - from genes to potential treatments.Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.Context-Dependent and Disease-Specific Diversity in Protein Interactions within Stress Granules.Recent insights into the cellular and molecular determinants of aging.Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay.Neurodegenerative disorders: Ataxin 2 reduction rescues motor defects.Oligonucleotide therapeutics in neurodegenerative diseases.Neurodegenerative disease: Two-for-one on potential therapies.CRISPR-Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity.TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD.Stress Granule Assembly Disrupts Nucleocytoplasmic Transport.Pathomechanisms of TDP-43 in neurodegeneration.Assisted delivery of antisense therapeutics in animal models of heritable neurodegenerative and neuromuscular disorders: a systematic review and meta-analysis.Antisense oligonucleotides in neurological disorders.RNA-Targeted Therapies and Amyotrophic Lateral Sclerosis.Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients Protein Phase Separation: A New Phase in Cell Biology Control of CNS functions by RNA-binding proteins in neurological diseases RNA-Binding Proteins in Amyotrophic Lateral Sclerosis Nuclear poly(ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis Conserved Pbp1/Ataxin-2 regulates retrotransposon activity and connects polyglutamine expansion-driven protein aggregation to lifespan-controlling rDNA repeats Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration Noncoding repeat expansions for ALS in Japan are associated with the gene These violent repeats have violent extends

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Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.

http://www.wikidata.org/entity/Q38288366

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2017年學術文章

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2017年學術文章

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2017年學術文章

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Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.

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Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.

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Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.

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Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.

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Armand Soriano

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Brenda Huang

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Gregor Bieri

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Hong Joo Kim

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James Messing

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Lindsay A Becker

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P2860

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

Ataxin-2-like is a regulator of stress granules and processing bodies

Altered ribostasis: RNA-protein granules in degenerative disorders

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

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

Treatment of infantile-onset spinal muscular atrophy with nusinersen: a phase 2, open-label, dose-escalation study.

Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue

Generation and characterization of Sca2 (ataxin-2) knockout mice

Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillization

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