polyglutamine

ataxin 3 ; Homo sapiens







175 Article(s)
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1 34938609 Allele-specific targeting of mutant ataxin-3 by antisense oligonucleotides in SCA3-iPSC-derived neurons. 2022 Mar 8 3
2 35386195 Plasma PolyQ-ATXN3 Levels Associate With Cerebellar Degeneration and Behavioral Abnormalities in a New AAV-Based SCA3 Mouse Model. 2022 2
3 35488942 Altered glucose metabolism and its association with carbonic anhydrase 8 in Machado-Joseph Disease. 2022 Apr 30 2
4 35587620 Comment on: Polyglutamine-Expanded Ataxin-3: A Target Engagement Marker for Spinocerebellar Ataxia Type 3 in Peripheral Blood. 2022 May 1
5 35587632 Reply to: "Comment on: Polyglutamine-Expanded Ataxin-3: A Target Engagement Marker for Spinocerebellar Ataxia Type 3 in Peripheral Blood". 2022 May 1
6 33065068 Capturing the Conformational Ensemble of the Mixed Folded Polyglutamine Protein Ataxin-3. 2021 Jan 7 1
7 33106888 PolyQ-expanded ataxin-3 protein levels in peripheral blood mononuclear cells correlate with clinical parameters in SCA3: a pilot study. 2021 Apr 5
8 33542212 A fine balance between Prpf19 and Exoc7 in achieving degradation of aggregated protein and suppression of cell death in spinocerebellar ataxia type 3. 2021 Feb 2 5
9 33629274 Mutant Ataxin-3-Containing Aggregates (MATAGGs) in Spinocerebellar Ataxia Type 3: Dynamics of the Disorder. 2021 Jul 3
10 33741019 Neurodegenerative phosphoprotein signaling landscape in models of SCA3. 2021 Mar 19 2
11 33837238 PolyQ-expanded proteins impair cellular proteostasis of ataxin-3 through sequestering the co-chaperone HSJ1 into aggregates. 2021 Apr 9 1
12 34199295 n-Butylidenephthalide Modulates Autophagy to Ameliorate Neuropathological Progress of Spinocerebellar Ataxia Type 3 through mTOR Pathway. 2021 Jun 13 6
13 34303201 Urine levels of the polyglutamine ataxin-3 protein are elevated in patients with spinocerebellar ataxia type 3. 2021 Aug 13
14 34345727 Clinical and genetic profile in index patients with spinocerebellar ataxia type 3 in Indonesia: case report. 2021 Jul 1
15 34397117 Polyglutamine-Expanded Ataxin-3: A Target Engagement Marker for Spinocerebellar Ataxia Type 3 in Peripheral Blood. 2021 Nov 4
16 34416891 Sodium valproate increases activity of the sirtuin pathway resulting in beneficial effects for spinocerebellar ataxia-3 in vivo. 2021 Aug 20 1
17 34535635 CRISPR/Cas9 mediated gene correction ameliorates abnormal phenotypes in spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cells. 2021 Sep 17 2
18 34685571 A Novel Calpain Inhibitor Compound Has Protective Effects on a Zebrafish Model of Spinocerebellar Ataxia Type 3. 2021 Sep 29 6
19 35111747 MicroRNA-409-3p Targeting at ATXN3 Reduces the Apoptosis of Dopamine Neurons Based on the Profile of miRNAs in the Cerebrospinal Fluid of Early Parkinson's Disease. 2021 1
20 31625269 The Machado-Joseph disease deubiquitylase ataxin-3 interacts with LC3C/GABARAP and promotes autophagy. 2020 Jan 2
21 31669734 Pathogenesis of SCA3 and implications for other polyglutamine diseases. 2020 Feb 1
22 31767406 Application of protein knockdown strategy targeting β-sheet structure to multiple disease-associated polyglutamine proteins. 2020 Jan 1 1
23 31783119 Druggable genome screen identifies new regulators of the abundance and toxicity of ATXN3, the Spinocerebellar Ataxia type 3 disease protein. 2020 Apr 5
24 31927329 Universal RNAi Triggers for the Specific Inhibition of Mutant Huntingtin, Atrophin-1, Ataxin-3, and Ataxin-7 Expression. 2020 Mar 6 2
25 31970864 The Machado-Joseph disease-associated form of ataxin-3 impacts dynamics of clathrin-coated pits. 2020 May 4
26 32274760 Machado-Joseph Disease: A Stress Combating Deubiquitylating Enzyme Changing Sides. 2020 6
27 32361312 Generation of human iPS cell line IBCHi002-A from spinocerebellar ataxia type 3/Machado-Joseph disease patient's fibroblasts. 2020 May 3
28 32643791 Degron capability of the hydrophobic C-terminus of the polyglutamine disease protein, ataxin-3. 2020 Oct 2
29 33087504 Toward allele-specific targeting therapy and pharmacodynamic marker for spinocerebellar ataxia type 3. 2020 Oct 21 10
30 33425926 Determining the Fate of Neurons in SCA3: ATX3, a Rising Decision Maker in Response to DNA Stresses and Beyond. 2020 4
31 30147021 Upregulation of miR-25 and miR-181 Family Members Correlates with Reduced Expression of ATXN3 in Lymphocytes from SCA3 Patients. 2019 7
32 30455355 Physiological and pathophysiological characteristics of ataxin-3 isoforms. 2019 Jan 11 2
33 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
34 31188927 State biomarkers for Machado Joseph disease: Validation, feasibility and responsiveness to change. 2019 2
35 31310802 Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3. 2019 Dec 2
36 31374463 Antisense oligonucleotide therapy rescues aggresome formation in a novel spinocerebellar ataxia type 3 human embryonic stem cell line. 2019 Aug 1
37 31378764 Disulfiram facilitates ataxin-3 nuclear translocation and potentiates the cytotoxicity in a cell model of SCA3. 2019 2
38 31683630 Removal of the Polyglutamine Repeat of Ataxin-3 by Redirecting pre-mRNA Processing. 2019 Oct 31 6
39 31687087 Pueraria lobata and Daidzein Reduce Cytotoxicity by Enhancing Ubiquitin-Proteasome System Function in SCA3-iPSC-Derived Neurons. 2019 2
40 31691128 From Pathogenesis to Novel Therapeutics for Spinocerebellar Ataxia Type 3: Evading Potholes on the Way to Translation. 2019 Oct 3
41 31828177 Development of an AAV-Based MicroRNA Gene Therapy to Treat Machado-Joseph Disease. 2019 Dec 13 1
42 29111377 Mass spectrometry analyses of normal and polyglutamine expanded ataxin-3 reveal novel interaction partners involved in mitochondrial function. 2018 Jan 8
43 29229556 Experimental and Clinical Strategies for Treating Spinocerebellar Ataxia Type 3. 2018 Feb 10 3
44 29334808 Identification of a novel site of interaction between ataxin-3 and the amyloid aggregation inhibitor polyglutamine binding peptide 1. 2018 Feb 6
45 29427106 The Neuropathology of Spinocerebellar Ataxia Type 3/Machado-Joseph Disease. 2018 2
46 29427109 Polyglutamine-Independent Features in Ataxin-3 Aggregation and Pathogenesis of Machado-Joseph Disease. 2018 1
47 29661116 CRISPR/Cas9-Targeted Deletion of Polyglutamine in Spinocerebellar Ataxia Type 3-Derived Induced Pluripotent Stem Cells. 2018 Jun 1 9
48 29972812 The Structural Properties in Solution of the Intrinsically Mixed Folded Protein Ataxin-3. 2018 Jul 3 1
49 30036587 The Machado-Joseph disease-associated expanded form of ataxin-3: Overexpression, purification, and preliminary biophysical and structural characterization. 2018 Dec 2
50 30042316 Protein Environment: A Crucial Triggering Factor in Josephin Domain Aggregation: The Role of 2,2,2-Trifluoroethanol. 2018 Jul 24 1