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Academic Journal

Targeting the VCP-binding motif of ataxin-3 improves phenotypes in Drosophila models of Spinocerebellar Ataxia Type 3.

  • Authors : Johnson SL; Department of Pharmacology, Wayne State University School of Medicine, Detroit, MI 48201, USA.; Libohova K

Subjects: Ataxin-3/Ataxin-3/Ataxin-3/*metabolism ; Machado-Joseph Disease/Machado-Joseph Disease/Machado-Joseph Disease/*metabolism ; Valosin Containing Protein/Valosin Containing Protein/Valosin Containing Protein/*metabolism

  • Source: Neurobiology of disease [Neurobiol Dis] 2021 Dec; Vol. 160, pp. 105516. Date of Electronic Publication: 2021 Sep 24.Publisher: Academic Press Country of Publication: United States NLM ID: 9500169 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Academic Journal

miRNA-Mediated Knockdown of ATXN3 Alleviates Molecular Disease Hallmarks in a Mouse Model for Spinocerebellar Ataxia Type 3.

  • Authors : Nobre RJ; Center for Neuroscience and Cell Biology (CNC), Molecular Therapy of Brain Disorders Group, University of Coimbra, Coimbra, Portugal.; Center for Innovative Biomedicine and Biotechnology (CIBB), Vectors, Gene and Cell Therapy Group, University of Coimbra, Coimbra, Portugal.

Subjects: Ataxin-3*/Ataxin-3*/Ataxin-3*/genetics ; Machado-Joseph Disease*/Machado-Joseph Disease*/Machado-Joseph Disease*/therapy ; MicroRNAs*/MicroRNAs*/MicroRNAs*/genetics

  • Source: Nucleic acid therapeutics [Nucleic Acid Ther] 2022 Jun; Vol. 32 (3), pp. 194-205. Date of Electronic Publication: 2021 Dec 07.Publisher: Mary Ann Liebert, Inc Country of Publication: United States NLM ID: 101562758 Publication Model: Print-Electronic Cited Medium: Internet

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Academic Journal

Pathophysiological interplay between O -GlcNAc transferase and the Machado-Joseph disease protein ataxin-3.

  • Authors : Pereira Sena P; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen 72076, Germany.; Center for Rare Diseases, University of Tübingen, Tübingen 72076, Germany.

Subjects: Ataxin-3/Ataxin-3/Ataxin-3/*metabolism ; Machado-Joseph Disease/Machado-Joseph Disease/Machado-Joseph Disease/*metabolism ; Machado-Joseph Disease/Machado-Joseph Disease/Machado-Joseph Disease/*pathology

  • Source: Proceedings of the National Academy of Sciences of the United States of America [Proc Natl Acad Sci U S A] 2021 Nov 23; Vol. 118 (47).Publisher: National Academy of Sciences Country of Publication: United States NLM ID: 7505876 Publication Model: Print Cited Medium: Internet ISSN:

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Academic Journal

PML-II recruits ataxin-3 to PML-NBs and inhibits its deubiquitinating activity.

  • Authors : Zhang Y; State Key Laboratory of Agrobiotechnology and College of Veterinary Medicine, China Agricultural University, Beijing, 100193, China.; Shao AW

Subjects: Ataxin-3/Ataxin-3/Ataxin-3/*metabolism ; Deubiquitinating Enzymes/Deubiquitinating Enzymes/Deubiquitinating Enzymes/*antagonists & inhibitors ; Intranuclear Inclusion Bodies/Intranuclear Inclusion Bodies/Intranuclear Inclusion Bodies/*metabolism

  • Source: Biochemical and biophysical research communications [Biochem Biophys Res Commun] 2021 May 21; Vol. 554, pp. 186-192. Date of Electronic Publication: 2021 Mar 30.Publisher: Elsevier Country of Publication: United States NLM ID: 0372516 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Academic Journal

PolyQ-expanded proteins impair cellular proteostasis of ataxin-3 through sequestering the co-chaperone HSJ1 into aggregates.

  • Authors : Yue HW; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, Shanghai, 200031, People's Republic of China.; University of Chinese Academy of Sciences, Beijing, 100049, People's Republic of China.

Subjects: Ataxin-3/Ataxin-3/Ataxin-3/*metabolism ; HSP40 Heat-Shock Proteins/HSP40 Heat-Shock Proteins/HSP40 Heat-Shock Proteins/*metabolism ; Molecular Chaperones/Molecular Chaperones/Molecular Chaperones/*metabolism

  • Source: Scientific reports [Sci Rep] 2021 Apr 09; Vol. 11 (1), pp. 7815. Date of Electronic Publication: 2021 Apr 09.Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN:

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Academic Journal

A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes.

  • Authors : Haas E; Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.; Centre for Rare Diseases, University of Tübingen, Tübingen, Germany.

Subjects: Disease Models, Animal* ; Phenotype*; Ataxin-3/Ataxin-3/Ataxin-3/*genetics

  • Source: Molecular neurobiology [Mol Neurobiol] 2022 Jan; Vol. 59 (1), pp. 495-522. Date of Electronic Publication: 2021 Oct 30.Publisher: Humana Press Country of Publication: United States NLM ID: 8900963 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Academic Journal

Poly(ADP-ribosyl)ation temporally confines SUMO-dependent ataxin-3 recruitment to control DNA double-strand break repair.

  • Authors : Pfeiffer A; Department of Cell and Molecular Biology, Karolinska Institutet, Biomedicum, Solnavägen 9, 17165 Stockholm, Sweden.; Herzog LK

Subjects: Ataxin-3*/Ataxin-3*/Ataxin-3*/genetics ; DNA Breaks, Double-Stranded* ; Poly ADP Ribosylation*

  • Source: Journal of cell science [J Cell Sci] 2021 Feb 08; Vol. 134 (3). Date of Electronic Publication: 2021 Feb 08.Publisher: Company of Biologists Country of Publication: England NLM ID: 0052457 Publication Model: Electronic Cited Medium: Internet ISSN:

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Academic Journal

Urine levels of the polyglutamine ataxin-3 protein are elevated in patients with spinocerebellar ataxia type 3.

  • Authors : Koike Y; Department of Neuroscience, Mayo Clinic, Jacksonville, FL, 32224, USA.; Jansen-West KR

Subjects: Ataxin-3/Ataxin-3/Ataxin-3/*urine ; Machado-Joseph Disease/Machado-Joseph Disease/Machado-Joseph Disease/*urine ; Peptides/Peptides/Peptides/*urine

  • Source: Parkinsonism & related disorders [Parkinsonism Relat Disord] 2021 Aug; Vol. 89, pp. 151-154. Date of Electronic Publication: 2021 Jul 17.Publisher: Elsevier Science Country of Publication: England NLM ID: 9513583 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Academic Journal

Capturing the Conformational Ensemble of the Mixed Folded Polyglutamine Protein Ataxin-3.

  • Authors : Sicorello A; The Maurice Wohl Clinical Neuroscience Institute, King's College London, 5 Cutcombe Road, SE5 9RT London, UK; UK Dementia Research Institute at King's College London, SE5 9RT London, UK.

Subjects: Protein Folding*; Ataxin-3/Ataxin-3/Ataxin-3/*chemistry ; Machado-Joseph Disease/Machado-Joseph Disease/Machado-Joseph Disease/*genetics

  • Source: Structure (London, England : 1993) [Structure] 2021 Jan 07; Vol. 29 (1), pp. 70-81.e5. Date of Electronic Publication: 2020 Oct 15.Publisher: Cell Press Country of Publication: United States NLM ID: 101087697 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Academic Journal

Ubiquitin-interacting motifs of ataxin-3 regulate its polyglutamine toxicity through Hsc70-4-dependent aggregation.

  • Authors : Johnson SL; Department of Pharmacology, Wayne State University, Detroit, United States.; Ranxhi B

Subjects: Ataxin-3*/Ataxin-3*/Ataxin-3*/chemistry ; Ataxin-3*/Ataxin-3*/Ataxin-3*/genetics ; Ataxin-3*/Ataxin-3*/Ataxin-3*/metabolism

  • Source: ELife [Elife] 2020 Sep 21; Vol. 9. Date of Electronic Publication: 2020 Sep 21.Publisher: eLife Sciences Publications, Ltd Country of Publication: England NLM ID: 101579614 Publication Model: Electronic Cited Medium: Internet

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  • 1-10 of  5,220 results for ""Ataxin-3""