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238 related items for PubMed ID: 24930601

  • 1. Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7.
    Ramachandran PS, Boudreau RL, Schaefer KA, La Spada AR, Davidson BL.
    Mol Ther; 2014 Sep; 22(9):1635-42. PubMed ID: 24930601
    [Abstract] [Full Text] [Related]

  • 2. Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7.
    Furrer SA, Waldherr SM, Mohanachandran MS, Baughn TD, Nguyen KT, Sopher BL, Damian VA, Garden GA, La Spada AR.
    Hum Mol Genet; 2013 Mar 01; 22(5):890-903. PubMed ID: 23197655
    [Abstract] [Full Text] [Related]

  • 3. Spinocerebellar ataxia type 7 cerebellar disease requires the coordinated action of mutant ataxin-7 in neurons and glia, and displays non-cell-autonomous bergmann glia degeneration.
    Furrer SA, Mohanachandran MS, Waldherr SM, Chang C, Damian VA, Sopher BL, Garden GA, La Spada AR.
    J Neurosci; 2011 Nov 09; 31(45):16269-78. PubMed ID: 22072678
    [Abstract] [Full Text] [Related]

  • 4. RNA interference-based therapy for spinocerebellar ataxia type 7 retinal degeneration.
    Ramachandran PS, Bhattarai S, Singh P, Boudreau RL, Thompson S, Laspada AR, Drack AV, Davidson BL.
    PLoS One; 2014 Nov 09; 9(4):e95362. PubMed ID: 24759684
    [Abstract] [Full Text] [Related]

  • 5. Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice.
    Chort A, Alves S, Marinello M, Dufresnois B, Dornbierer JG, Tesson C, Latouche M, Baker DP, Barkats M, El Hachimi KH, Ruberg M, Janer A, Stevanin G, Brice A, Sittler A.
    Brain; 2013 Jun 09; 136(Pt 6):1732-45. PubMed ID: 23518714
    [Abstract] [Full Text] [Related]

  • 6. Broad therapeutic benefit after RNAi expression vector delivery to deep cerebellar nuclei: implications for spinocerebellar ataxia type 1 therapy.
    Keiser MS, Boudreau RL, Davidson BL.
    Mol Ther; 2014 Mar 09; 22(3):588-595. PubMed ID: 24419082
    [Abstract] [Full Text] [Related]

  • 7. SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types.
    Yvert G, Lindenberg KS, Devys D, Helmlinger D, Landwehrmeyer GB, Mandel JL.
    Hum Mol Genet; 2001 Aug 01; 10(16):1679-92. PubMed ID: 11487572
    [Abstract] [Full Text] [Related]

  • 8. AAV-Mediated CAG-Targeting Selectively Reduces Polyglutamine-Expanded Protein and Attenuates Disease Phenotypes in a Spinocerebellar Ataxia Mouse Model.
    Niewiadomska-Cimicka A, Fievet L, Surdyka M, Jesion E, Keime C, Singer E, Eisenmann A, Kalinowska-Poska Z, Nguyen HHP, Fiszer A, Figiel M, Trottier Y.
    Int J Mol Sci; 2024 Apr 15; 25(8):. PubMed ID: 38673939
    [Abstract] [Full Text] [Related]

  • 9. Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.
    Garden GA, Libby RT, Fu YH, Kinoshita Y, Huang J, Possin DE, Smith AC, Martinez RA, Fine GC, Grote SK, Ware CB, Einum DD, Morrison RS, Ptacek LJ, Sopher BL, La Spada AR.
    J Neurosci; 2002 Jun 15; 22(12):4897-905. PubMed ID: 12077187
    [Abstract] [Full Text] [Related]

  • 10. SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity.
    Yoo SY, Pennesi ME, Weeber EJ, Xu B, Atkinson R, Chen S, Armstrong DL, Wu SM, Sweatt JD, Zoghbi HY.
    Neuron; 2003 Feb 06; 37(3):383-401. PubMed ID: 12575948
    [Abstract] [Full Text] [Related]

  • 11. Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model.
    Helmlinger D, Abou-Sleymane G, Yvert G, Rousseau S, Weber C, Trottier Y, Mandel JL, Devys D.
    J Neurosci; 2004 Feb 25; 24(8):1881-7. PubMed ID: 14985428
    [Abstract] [Full Text] [Related]

  • 12. Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration.
    Palhan VB, Chen S, Peng GH, Tjernberg A, Gamper AM, Fan Y, Chait BT, La Spada AR, Roeder RG.
    Proc Natl Acad Sci U S A; 2005 Jun 14; 102(24):8472-7. PubMed ID: 15932940
    [Abstract] [Full Text] [Related]

  • 13. Antisense oligonucleotides targeting mutant Ataxin-7 restore visual function in a mouse model of spinocerebellar ataxia type 7.
    Niu C, Prakash TP, Kim A, Quach JL, Huryn LA, Yang Y, Lopez E, Jazayeri A, Hung G, Sopher BL, Brooks BP, Swayze EE, Bennett CF, La Spada AR.
    Sci Transl Med; 2018 Oct 31; 10(465):. PubMed ID: 30381411
    [Abstract] [Full Text] [Related]

  • 14. Overexpression of HGF attenuates the degeneration of Purkinje cells and Bergmann glia in a knockin mouse model of spinocerebellar ataxia type 7.
    Noma S, Ohya-Shimada W, Kanai M, Ueda K, Nakamura T, Funakoshi H.
    Neurosci Res; 2012 Jun 31; 73(2):115-21. PubMed ID: 22426494
    [Abstract] [Full Text] [Related]

  • 15. Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice.
    Yvert G, Lindenberg KS, Picaud S, Landwehrmeyer GB, Sahel JA, Mandel JL.
    Hum Mol Genet; 2000 Oct 12; 9(17):2491-506. PubMed ID: 11030754
    [Abstract] [Full Text] [Related]

  • 16. Design of RNAi hairpins for mutation-specific silencing of ataxin-7 and correction of a SCA7 phenotype.
    Scholefield J, Greenberg LJ, Weinberg MS, Arbuthnot PB, Abdelgany A, Wood MJ.
    PLoS One; 2009 Sep 30; 4(9):e7232. PubMed ID: 19789634
    [Abstract] [Full Text] [Related]

  • 17. Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins.
    Alves S, Marais T, Biferi MG, Furling D, Marinello M, El Hachimi K, Cartier N, Ruberg M, Stevanin G, Brice A, Barkats M, Sittler A.
    Mol Neurodegener; 2016 Jul 28; 11(1):58. PubMed ID: 27465358
    [Abstract] [Full Text] [Related]

  • 18. A role for both wild-type and expanded ataxin-7 in transcriptional regulation.
    Ström AL, Forsgren L, Holmberg M.
    Neurobiol Dis; 2005 Dec 28; 20(3):646-55. PubMed ID: 15936949
    [Abstract] [Full Text] [Related]

  • 19. Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration.
    Garden GA, La Spada AR.
    Cerebellum; 2008 Dec 28; 7(2):138-49. PubMed ID: 18418675
    [Abstract] [Full Text] [Related]

  • 20. Molecular Targets and Therapeutic Strategies in Spinocerebellar Ataxia Type 7.
    Niewiadomska-Cimicka A, Trottier Y.
    Neurotherapeutics; 2019 Oct 28; 16(4):1074-1096. PubMed ID: 31432449
    [Abstract] [Full Text] [Related]

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