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CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome

Neuron (Cambridge, Mass.), 2013-05, Vol.78 (3), p.440-455

2013

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Pathogenesis of SCA3 and implications for other polyglutamine diseases

Neurobiology of disease, 2020-02, Vol.134, p.104635-104635, Article 104635

2020

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RNA-mediated neurodegeneration in repeat expansion disorders

Annals of neurology, 2010-03, Vol.67 (3), p.291-300

2010

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Alzheimer's disease and environmental exposure to lead: the epidemiologic evidence and potential role of epigenetics

Current Alzheimer research, 2012-06, Vol.9 (5), p.563

2012

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Ube2w and Ataxin-3 Coordinately Regulate the Ubiquitin Ligase CHIP

Molecular cell, 2011-08, Vol.43 (4), p.599-612

2011

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Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice

Annals of neurology, 2018-07, Vol.84 (1), p.64-77

2018

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Peri-Infarct Upregulation of the Oxytocin Receptor in Vascular Dementia

Journal of neuropathology and experimental neurology, 2019-05, Vol.78 (5), p.436-452

2019

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Associations of healthy lifestyle and socioeconomic status with cognitive function in U.S. older adults

Scientific reports, 2023-05, Vol.13 (1), p.7513-7513, Article 7513

2023

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Accelerated neurodegeneration through chaperone-mediated oligomerization of tau

The Journal of clinical investigation, 2013-10, Vol.123 (10), p.4158-4169

2013

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RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome

Human molecular genetics, 2015-08, Vol.24 (15), p.4317-4326

2015

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Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3

The Journal of neuroscience, 2011-09, Vol.31 (36), p.13002-13014

2011

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Genome-wide DNA methylation differences between late-onset Alzheimer's disease and cognitively normal controls in human frontal cortex

Journal of Alzheimer's disease, 2012-01, Vol.29 (3), p.571

2012

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Loss of the Spinocerebellar Ataxia type 3 disease protein ATXN3 alters transcription of multiple signal transduction pathways

PloS one, 2018-09, Vol.13 (9), p.e0204438-e0204438

2018

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Wild-type and pathogenic forms of ubiquilin 2 differentially modulate components of the autophagy-lysosome pathways

Journal of pharmacological sciences, 2023-07, Vol.152 (3), p.182-192

2023

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Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models

Molecular therapy. Nucleic acids, 2017-06, Vol.7 (C), p.200-210

2017

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RNA Interference Improves Motor and Neuropathological Abnormalities in a Huntington's Disease Mouse Model

Proceedings of the National Academy of Sciences - PNAS, 2005-04, Vol.102 (16), p.5820-5825

2005

Volltextzugriff (PDF)

Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3

Neurobiology of disease, 2019-12, Vol.132, p.104535-104535, Article 104535

2019

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JosD1, a Membrane-targeted Deubiquitinating Enzyme, Is Activated by Ubiquitination and Regulates Membrane Dynamics, Cell Motility, and Endocytosis

The Journal of biological chemistry, 2013-06, Vol.288 (24), p.17145-17155

2013

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Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly

Proceedings of the National Academy of Sciences - PNAS, 2018-10, Vol.115 (44), p.E10495-E10504

2018

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Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties

PloS one, 2010-10, Vol.5 (10), p.e13695

2010

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The Machado―Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability

Human molecular genetics, 2011-01, Vol.20 (1), p.141-154

2011

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Spinocerebellar ataxias: prospects and challenges for therapy development

Nature reviews. Neurology, 2018-10, Vol.14 (10), p.590

2018

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The Deubiquitinating Enzyme Ataxin-3, a Polyglutamine Disease Protein, Edits Lys63 Linkages in Mixed Linkage Ubiquitin Chains

The Journal of biological chemistry, 2008-09, Vol.283 (39), p.26436-26443

2008

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Toward RNAi Therapy for the Polyglutamine Disease Machado–Joseph Disease

Molecular therapy, 2013-10, Vol.21 (10), p.1898-1908

2013

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Regional and age-dependent changes in ubiquitination in cellular and mouse models of spinocerebellar ataxia type 3

Frontiers in molecular neuroscience, 2023-04, Vol.16, p.1154203-1154203

2023

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CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome

Pathogenesis of SCA3 and implications for other polyglutamine diseases

RNA-mediated neurodegeneration in repeat expansion disorders

Alzheimer's disease and environmental exposure to lead: the epidemiologic evidence and potential role of epigenetics

Ube2w and Ataxin-3 Coordinately Regulate the Ubiquitin Ligase CHIP

Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice

Peri-Infarct Upregulation of the Oxytocin Receptor in Vascular Dementia

Associations of healthy lifestyle and socioeconomic status with cognitive function in U.S. older adults

Accelerated neurodegeneration through chaperone-mediated oligomerization of tau

RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome

Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3

Genome-wide DNA methylation differences between late-onset Alzheimer's disease and cognitively normal controls in human frontal cortex

Loss of the Spinocerebellar Ataxia type 3 disease protein ATXN3 alters transcription of multiple signal transduction pathways

Wild-type and pathogenic forms of ubiquilin 2 differentially modulate components of the autophagy-lysosome pathways

Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models

RNA Interference Improves Motor and Neuropathological Abnormalities in a Huntington's Disease Mouse Model

Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3

JosD1, a Membrane-targeted Deubiquitinating Enzyme, Is Activated by Ubiquitination and Regulates Membrane Dynamics, Cell Motility, and Endocytosis

Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly

Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties

The Machado―Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability

Spinocerebellar ataxias: prospects and challenges for therapy development

The Deubiquitinating Enzyme Ataxin-3, a Polyglutamine Disease Protein, Edits Lys63 Linkages in Mixed Linkage Ubiquitin Chains

Toward RNAi Therapy for the Polyglutamine Disease Machado–Joseph Disease

Regional and age-dependent changes in ubiquitination in cellular and mouse models of spinocerebellar ataxia type 3

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