Axonal inclusions in spinocerebellar ataxia type 3. - Wikidata - awgldk - TriplyDB

Axonal inclusions in spinocerebellar ataxia type 3.

Axonal inclusions in spinocerebellar ataxia type 3.

http://www.wikidata.org/entity/Q34072128

about

Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathways Blood RNA biomarkers in prodromal PARK4 and rapid eye movement sleep behavior disorder show role of complexin 1 loss for risk of Parkinson's disease.Huntington's disease (HD): degeneration of select nuclei, widespread occurrence of neuronal nuclear and axonal inclusions in the brainstem.Brain pathology of spinocerebellar ataxias.p62/sequestosome 1 regulates aggresome formation of pathogenic ataxin-3 with expanded polyglutamine Sequestration of cellular interacting partners by protein aggregates: implication in a loss-of-function pathology.A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript.Change in the cortical complexity of spinocerebellar ataxia type 3 appears earlier than clinical symptoms Toward understanding Machado-Joseph disease Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study.Autophagy in axonal and dendritic degeneration.Autophagy Promoted the Degradation of Mutant ATXN3 in Neurally Differentiated Spinocerebellar Ataxia-3 Human Induced Pluripotent Stem Cells.Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease.Aggregation of polyglutamine-expanded ataxin-3 sequesters its specific interacting partners into inclusions: implication in a loss-of-function pathology.Axonal degeneration as a therapeutic target in the CNS.Nonmotor and extracerebellar features in Machado-Joseph disease: a review.Ataxin-3 protein and RNA toxicity in spinocerebellar ataxia type 3: current insights and emerging therapeutic strategies.From pathways to targets: understanding the mechanisms behind polyglutamine disease.Pathology and function of nuclear amyloid. Protein homeostasis matters.Peripheral neuropathy and parkinsonism: a large clinical and pathogenic spectrum.Overexpression of cystathionine γ-lyase suppresses detrimental effects of spinocerebellar ataxia type 3.Bcl-2 Decreases the Affinity of SQSTM1/p62 to Poly-Ubiquitin Chains and Suppresses the Aggregation of Misfolded Protein in Neurodegenerative Disease.Localized vs. Systematic Neurodegeneration: A Paradigm Shift in Understanding Neurodegenerative Diseases.Caffeine and adenosine A(2A) receptor inactivation decrease striatal neuropathology in a lentiviral-based model of Machado-Joseph disease.Neuropathological diagnostic considerations in hyperkinetic movement disorders.Cerebellar cognitive affective syndrome in Machado Joseph disease: core clinical features.Accumulation of phosphorylated TDP-43 in the CNS of a patient with Cockayne syndrome.Polyglutamine aggregation in Huntington's disease and spinocerebellar ataxia type 3: similar mechanisms in aggregate formation.Deregulation of autophagy in postmortem brains of Machado-Joseph disease patients.Genotype-phenotype correlations in spastic paraplegia type 7: a study in a large Dutch cohort.On the distribution of intranuclear and cytoplasmic aggregates in the brainstem of patients with spinocerebellar ataxia type 2 and 3.Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3).Spinocerebellar ataxia type 2 (SCA2) is associated with TDP-43 pathology.Elavl3 is essential for the maintenance of Purkinje neuron axons.Spinocerebellar ataxia type 2 (SCA2): identification of early brain degeneration in one monozygous twin in the initial disease stage.

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P2860

Axonal inclusions in spinocerebellar ataxia type 3.

http://www.wikidata.org/entity/Q34072128

description

2010 nî lūn-bûn

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2010 թուականի Յուլիսին հրատարակուած գիտական յօդուած

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2010 թվականի հուլիսին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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Axonal inclusions in spinocerebellar ataxia type 3.

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Axonal inclusions in spinocerebellar ataxia type 3.

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Axonal inclusions in spinocerebellar ataxia type 3.

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Axonal inclusions in spinocerebellar ataxia type 3.

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Axonal inclusions in spinocerebellar ataxia type 3.

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Acta Neuropathologica

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Axonal inclusions in spinocerebellar ataxia type 3.

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Christian Schultz

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Ewout R Brunt

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Harm H Kampinga

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Henry Paulson

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Kay Seidel

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Rob A de Vos

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Stefanie Frank

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Udo Rüb

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Wilfred F A den Dunnen

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P2860

Structure of the ubiquitin-associated domain of p62 (SQSTM1) and implications for mutations that cause Paget's disease of bone

Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain

Ubiquitin-binding protein p62 is present in neuronal and glial inclusions in human tauopathies and synucleinopathies

CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

Autosomal dominant cerebellar ataxia: phenotypic differences in genetically defined subtypes?

Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence

Trinucleotide Repeat Disorders

p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

The ubiquitin-proteasome pathway: on protein death and cell life

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