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34273111 | Identification of the calpain-generated toxic fragment of ataxin-3 protein provides new avenues for therapy of Machado-Joseph disease| Spinocerebellar ataxia type 3. | 2022 Feb |
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34298131 | ULK overexpression mitigates motor deficits and neuropathology in mouse models of Machado-Joseph disease. | 2022 Jan 5 |
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34716557 | A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes. | 2022 Jan |
3 |
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35042771 | Impaired Oligodendrocyte Maturation Is an Early Feature in SCA3 Disease Pathogenesis. | 2022 Feb 23 |
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35386195 | Plasma PolyQ-ATXN3 Levels Associate With Cerebellar Degeneration and Behavioral Abnormalities in a New AAV-Based SCA3 Mouse Model. | 2022 |
10 |
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35605759 | Altered retinal structure and function in Spinocerebellar ataxia type 3. | 2022 Aug |
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33741019 | Neurodegenerative phosphoprotein signaling landscape in models of SCA3. | 2021 Mar 19 |
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| 8 |
34199295 | n-Butylidenephthalide Modulates Autophagy to Ameliorate Neuropathological Progress of Spinocerebellar Ataxia Type 3 through mTOR Pathway. | 2021 Jun 13 |
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| 9 |
34220448 | Broad Influence of Mutant Ataxin-3 on the Proteome of the Adult Brain, Young Neurons, and Axons Reveals Central Molecular Processes and Biomarkers in SCA3/MJD Using Knock-In Mouse Model. | 2021 |
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31960910 | Calpain-1 ablation partially rescues disease-associated hallmarks in models of Machado-Joseph disease. | 2020 Apr 15 |
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| 11 |
32867861 | The blood-brain barrier is disrupted in Machado-Joseph disease/spinocerebellar ataxia type 3: evidence from transgenic mice and human post-mortem samples. | 2020 Aug 31 |
3 |
| 12 |
33411688 | Polyglutamine-expanded ataxin3 alter specific gene expressions through changing DNA methylation status in SCA3/MJD. | 2020 Dec 19 |
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30343032 | Pharmacological enhancement of retinoid-related orphan receptor α function mitigates spinocerebellar ataxia type 3 pathology. | 2019 Jan |
2 |
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30760052 | RNA Interference Therapy for Machado-Joseph Disease: Long-Term Safety Profile of Lentiviral Vectors Encoding Short Hairpin RNAs Targeting Mutant Ataxin-3. | 2019 Jul |
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| 15 |
31157458 | Protective roles of carbonic anhydrase 8 in Machado-Joseph Disease. | 2019 Oct |
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| 16 |
31394429 | Suppression of Mutant Protein Expression in SCA3 and SCA1 Mice Using a CAG Repeat-Targeting Antisense Oligonucleotide. | 2019 Sep 6 |
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| 17 |
31666698 | Allele-selective lowering of mutant HTT protein by HTT-LC3 linker compounds. | 2019 Nov |
4 |
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29476013 | Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3. | 2018 Mar 13 |
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| 19 |
29908063 | Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice. | 2018 Jul |
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| 20 |
29929540 | Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model. | 2018 Jun 22 |
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30231063 | Loss of the Spinocerebellar Ataxia type 3 disease protein ATXN3 alters transcription of multiple signal transduction pathways. | 2018 |
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28032667 | Caffeine alleviates progressive motor deficits in a transgenic mouse model of spinocerebellar ataxia. | 2017 Mar |
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28445460 | Polyglutamine tracts regulate beclin 1-dependent autophagy. | 2017 May 4 |
9 |
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28624196 | Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models. | 2017 Jun 16 |
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28854700 | Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes. | 2017 Sep 1 |
3 |
| 26 |
28918024 | Antisense Oligonucleotide-Mediated Removal of the Polyglutamine Repeat in Spinocerebellar Ataxia Type 3 Mice. | 2017 Sep 15 |
3 |
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26990650 | In vivo assessment of riluzole as a potential therapeutic drug for spinocerebellar ataxia type 3. | 2016 Jul |
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27165717 | Caloric restriction blocks neuropathology and motor deficits in Machado-Joseph disease mouse models through SIRT1 pathway. | 2016 May 11 |
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27851749 | The Machado-Joseph Disease Deubiquitinase Ataxin-3 Regulates the Stability and Apoptotic Function of p53. | 2016 Nov |
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25143392 | Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells. | 2015 Jan 1 |
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25320121 | A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript. | 2015 Mar 1 |
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26254860 | T1-11 and JMF1907 ameliorate polyglutamine-expanded ataxin-3-induced neurodegeneration, transcriptional dysregulation and ataxic symptom in the SCA3 transgenic mouse. | 2015 Dec |
5 |
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23955261 | Mutant ataxin-3 with an abnormally expanded polyglutamine chain disrupts dendritic development and metabotropic glutamate receptor signaling in mouse cerebellar Purkinje cells. | 2014 Feb |
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| 34 |
24817574 | Calpain inhibition reduces ataxin-3 cleavage alleviating neuropathology and motor impairments in mouse models of Machado-Joseph disease. | 2014 Sep 15 |
2 |
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25139423 | Polyglutamine-expanded ataxin-3 impairs long-term depression in Purkinje neurons of SCA3 transgenic mouse by inhibiting HAT and impairing histone acetylation. | 2014 Oct 2 |
4 |
| 36 |
25144231 | RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph disease. | 2014 |
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23100324 | Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3). | 2013 Feb 1 |
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23347954 | H1152 promotes the degradation of polyglutamine-expanded ataxin-3 or ataxin-7 independently of its ROCK-inhibiting effect and ameliorates mutant ataxin-3-induced neurodegeneration in the SCA3 transgenic mouse. | 2013 Jul |
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23626768 | Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice. | 2013 |
3 |
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23653622 | Ataxin-3 and its e3 partners: implications for machado-joseph disease. | 2013 |
1 |
| 41 |
23765441 | Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease. | 2013 Oct |
1 |
| 42 |
23801739 | Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease. | 2013 Jul |
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23820820 | Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice. | 2013 Oct |
2 |
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24683430 | The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease. | 2013 |
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22037589 | Compromised mitochondrial complex II in models of Machado-Joseph disease. | 2012 Feb |
2 |
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22843411 | Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease. | 2012 Aug |
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20940148 | The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability. | 2011 Jan 1 |
4 |
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21047555 | HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3. | 2011 Feb |
2 |
| 49 |
21092747 | p53 activation mediates polyglutamine-expanded ataxin-3 upregulation of Bax expression in cerebellar and pontine nuclei neurons. | 2011 Feb |
6 |
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21944750 | Expanded polyglutamine-binding peptoid as a novel therapeutic agent for treatment of Huntington's disease. | 2011 Sep 23 |
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