| 1 |
34906452 | Digenic inheritance of STUB1 variants and TBP polyglutamine expansions explains the incomplete penetrance of SCA17 and SCA48. | 2022 Jan |
2 |
| 2 |
35401096 | The Molecular Basis of Spinocerebellar Ataxia Type 7. | 2022 |
4 |
| 3 |
35422034 | Functional characterization of variants of unknown significance in a spinocerebellar ataxia patient using an unsupervised machine learning pipeline. | 2022 Apr 14 |
2 |
| 4 |
33338633 | Polyglutamine expanded Ataxin-7 induces DNA damage and alters FUS localization and function. | 2021 Jan |
7 |
| 5 |
33626063 | Molecular and electrophysiological features of spinocerebellar ataxia type seven in induced pluripotent stem cells. | 2021 |
3 |
| 6 |
33837238 | PolyQ-expanded proteins impair cellular proteostasis of ataxin-3 through sequestering the co-chaperone HSJ1 into aggregates. | 2021 Apr 9 |
1 |
| 7 |
31767406 | Application of protein knockdown strategy targeting β-sheet structure to multiple disease-associated polyglutamine proteins. | 2020 Jan 1 |
1 |
| 8 |
31927329 | Universal RNAi Triggers for the Specific Inhibition of Mutant Huntingtin, Atrophin-1, Ataxin-3, and Ataxin-7 Expression. | 2020 Mar 6 |
2 |
| 9 |
32518289 | Author Correction: Structural and dynamic studies reveal that the Ala-rich region of ataxin-7 initiates α-helix formation of the polyQ tract but suppresses its aggregation. | 2020 Jun 9 |
1 |
| 10 |
32581673 | The Mechanisms of Nuclear Proteotoxicity in Polyglutamine Spinocerebellar Ataxias. | 2020 |
2 |
| 11 |
32581696 | Gene Deregulation and Underlying Mechanisms in Spinocerebellar Ataxias With Polyglutamine Expansion. | 2020 |
2 |
| 12 |
32962458 | Polyglutamine spinocerebellar ataxias: emerging therapeutic targets. | 2020 Nov |
4 |
| 13 |
30559154 | SUMOylation by SUMO2 is implicated in the degradation of misfolded ataxin-7 via RNF4 in SCA7 models. | 2019 Jan 11 |
10 |
| 14 |
30699348 | Metabolic and Organelle Morphology Defects in Mice and Human Patients Define Spinocerebellar Ataxia Type 7 as a Mitochondrial Disease. | 2019 Jan 29 |
3 |
| 15 |
30707359 | EvoPPI 1.0: a Web Platform for Within- and Between-Species Multiple Interactome Comparisons and Application to Nine PolyQ Proteins Determining Neurodegenerative Diseases. | 2019 Mar |
2 |
| 16 |
31097749 | Structural and dynamic studies reveal that the Ala-rich region of ataxin-7 initiates α-helix formation of the polyQ tract but suppresses its aggregation. | 2019 May 16 |
3 |
| 17 |
31317427 | Molecular Mechanisms and Therapeutics for SCA17. | 2019 Oct |
6 |
| 18 |
31348003 | Ataxin-7 and Non-stop coordinate SCAR protein levels, subcellular localization, and actin cytoskeleton organization. | 2019 Jul 26 |
3 |
| 19 |
31432449 | Molecular Targets and Therapeutic Strategies in Spinocerebellar Ataxia Type 7. | 2019 Oct |
3 |
| 20 |
29427104 | Molecular Mechanisms and Therapeutic Strategies in Spinocerebellar Ataxia Type 7. | 2018 |
5 |
| 21 |
29876803 | Oxidative Stress in Spinocerebellar Ataxia Type 7 Is Associated with Disease Severity. | 2018 Oct |
2 |
| 22 |
30473770 | Recent advances in understanding dominant spinocerebellar ataxias from clinical and genetic points of view. | 2018 |
4 |
| 23 |
28774347 | microRNA dysregulation in polyglutamine toxicity of TATA-box binding protein is mediated through STAT1 in mouse neuronal cells. | 2017 Aug 3 |
2 |
| 24 |
28821675 | Synergistic Toxicity of Polyglutamine-Expanded TATA-Binding Protein in Glia and Neuronal Cells: Therapeutic Implications for Spinocerebellar Ataxia 17. | 2017 Sep 20 |
5 |
| 25 |
28032013 | Molecular mechanisms underlying Spinocerebellar Ataxia 17 (SCA17) pathogenesis. | 2016 |
4 |
| 26 |
25647692 | Altered p53 and NOX1 activity cause bioenergetic defects in a SCA7 polyglutamine disease model. | 2015 Apr-May |
4 |
| 27 |
25800379 | The potential of lactulose and melibiose, two novel trehalase-indigestible and autophagy-inducing disaccharides, for polyQ-mediated neurodegenerative disease treatment. | 2015 May |
2 |
| 28 |
26195632 | Aggregation of Polyglutamine-expanded Ataxin 7 Protein Specifically Sequesters Ubiquitin-specific Protease 22 and Deteriorates Its Deubiquitinating Function in the Spt-Ada-Gcn5-Acetyltransferase (SAGA) Complex. | 2015 Sep 4 |
12 |
| 29 |
25054097 | Pulling complexes out of complex diseases: Spinocerebellar Ataxia 7. | 2014 |
5 |
| 30 |
25342886 | Indole and synthetic derivative activate chaperone expression to reduce polyQ aggregation in SCA17 neuronal cell and slice culture models. | 2014 |
4 |
| 31 |
23592174 | Inhibition of autophagy via p53-mediated disruption of ULK1 in a SCA7 polyglutamine disease model. | 2013 Jul |
2 |
| 32 |
23699518 | A novel transgenic rat model for spinocerebellar ataxia type 17 recapitulates neuropathological changes and supplies in vivo imaging biomarkers. | 2013 May 22 |
2 |
| 33 |
23892081 | Structural basis for recognition of the third SH3 domain of full-length R85 (R85FL)/ponsin by ataxin-7. | 2013 Sep 17 |
5 |
| 34 |
24129567 | Direct inhibition of Gcn5 protein catalytic activity by polyglutamine-expanded ataxin-7. | 2013 Nov 22 |
10 |
| 35 |
22367614 | Differential degradation of full-length and cleaved ataxin-7 fragments in a novel stable inducible SCA7 model. | 2012 Jun |
2 |
| 36 |
22664922 | Regulation of BACE1 by miR-29a/b in a cellular model of Spinocerebellar Ataxia 17. | 2012 Jun |
2 |
| 37 |
22684686 | Brain pathology of spinocerebellar ataxias. | 2012 Jul |
2 |
| 38 |
22827889 | Expanded ataxin-7 cause toxicity by inducing ROS production from NADPH oxidase complexes in a stable inducible Spinocerebellar ataxia type 7 (SCA7) model. | 2012 Jul 24 |
5 |
| 39 |
23236151 | Reelin is a target of polyglutamine expanded ataxin-7 in human spinocerebellar ataxia type 7 (SCA7) astrocytes. | 2012 Dec 26 |
8 |
| 40 |
20732423 | Amyloid precursor-like protein 2 cleavage contributes to neuronal intranuclear inclusions and cytotoxicity in spinocerebellar ataxia-7 (SCA7). | 2011 Jan |
6 |
| 41 |
21078624 | Comparison of an expanded ataxia interactome with patient medical records reveals a relationship between macular degeneration and ataxia. | 2011 Feb 1 |
4 |
| 42 |
21653638 | A Drosophila model of the neurodegenerative disease SCA17 reveals a role of RBP-J/Su(H) in modulating the pathological outcome. | 2011 Sep 1 |
3 |
| 43 |
19843541 | SUMOylation attenuates the aggregation propensity and cellular toxicity of the polyglutamine expanded ataxin-7. | 2010 Jan 1 |
5 |
| 44 |
19955365 | Posttranslational modification of ataxin-7 at lysine 257 prevents autophagy-mediated turnover of an N-terminal caspase-7 cleavage fragment. | 2009 Dec 2 |
6 |
| 45 |
20030245 | [Molecular genetic approach to spinocerebellar ataxias]. | 2009 Nov |
2 |
| 46 |
17971076 | Spinocerebellar ataxia type 7 (SCA7): widespread brain damage in an adult-onset patient with progressive visual impairments in comparison with an adult-onset patient without visual impairments. | 2008 Apr |
2 |
| 47 |
18043721 | Instability of expanded CAG/CAA repeats in spinocerebellar ataxia type 17. | 2008 Feb |
1 |
| 48 |
18418675 | Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration. | 2008 |
10 |
| 49 |
18418687 | Spinocerebellar ataxia 17 (SCA17) and Huntington's disease-like 4 (HDL4). | 2008 |
2 |
| 50 |
17005371 | Polyglutamine-expanded ataxin-7 decreases nuclear translocation of NF-kappaB p65 and impairs NF-kappaB activity by inhibiting proteasome activity of cerebellar neurons. | 2007 Mar |
3 |