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Journal Abstract Search


380 related items for PubMed ID: 22072678

  • 1. 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]

  • 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. 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 01; 73(2):115-21. PubMed ID: 22426494
    [Abstract] [Full Text] [Related]

  • 4. Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration.
    Garden GA, La Spada AR.
    Cerebellum; 2008 Jun 01; 7(2):138-49. PubMed ID: 18418675
    [Abstract] [Full Text] [Related]

  • 5. 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]

  • 6. 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 15; 22(9):1635-42. PubMed ID: 24930601
    [Abstract] [Full Text] [Related]

  • 7. 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 15; 136(Pt 6):1732-45. PubMed ID: 23518714
    [Abstract] [Full Text] [Related]

  • 8. Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport.
    Custer SK, Garden GA, Gill N, Rueb U, Libby RT, Schultz C, Guyenet SJ, Deller T, Westrum LE, Sopher BL, La Spada AR.
    Nat Neurosci; 2006 Oct 15; 9(10):1302-11. PubMed ID: 16936724
    [Abstract] [Full Text] [Related]

  • 9. 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]

  • 10. 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]

  • 11. Altered calcium signaling in Bergmann glia contributes to spinocerebellar ataxia type-1 in a mouse model of SCA1.
    Nanclares C, Noriega-Prieto JA, Labrada-Moncada FE, Cvetanovic M, Araque A, Kofuji P.
    Neurobiol Dis; 2023 Oct 15; 187():106318. PubMed ID: 37802154
    [Abstract] [Full Text] [Related]

  • 12. 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]

  • 13. Posttranslational modification of ataxin-7 at lysine 257 prevents autophagy-mediated turnover of an N-terminal caspase-7 cleavage fragment.
    Mookerjee S, Papanikolaou T, Guyenet SJ, Sampath V, Lin A, Vitelli C, DeGiacomo F, Sopher BL, Chen SF, La Spada AR, Ellerby LM.
    J Neurosci; 2009 Dec 02; 29(48):15134-44. PubMed ID: 19955365
    [Abstract] [Full Text] [Related]

  • 14. A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD.
    Switonski PM, Szlachcic WJ, Krzyzosiak WJ, Figiel M.
    Neurobiol Dis; 2015 Jan 02; 73():174-88. PubMed ID: 25301414
    [Abstract] [Full Text] [Related]

  • 15. Differential effects of Wnt-β-catenin signaling in Purkinje cells and Bergmann glia in spinocerebellar ataxia type 1.
    Luttik K, Tejwani L, Ju H, Driessen T, Smeets CJLM, Edamakanti CR, Khan A, Yun J, Opal P, Lim J.
    Proc Natl Acad Sci U S A; 2022 Aug 23; 119(34):e2208513119. PubMed ID: 35969780
    [Abstract] [Full Text] [Related]

  • 16. 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]

  • 17. 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]

  • 18. Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation.
    Young JE, Gouw L, Propp S, Sopher BL, Taylor J, Lin A, Hermel E, Logvinova A, Chen SF, Chen S, Bredesen DE, Truant R, Ptacek LJ, La Spada AR, Ellerby LM.
    J Biol Chem; 2007 Oct 12; 282(41):30150-60. PubMed ID: 17646170
    [Abstract] [Full Text] [Related]

  • 19. 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 Oct 12; 9(4):e95362. PubMed ID: 24759684
    [Abstract] [Full Text] [Related]

  • 20. Molecular Targets and Therapeutic Strategies in Spinocerebellar Ataxia Type 7.
    Niewiadomska-Cimicka A, Trottier Y.
    Neurotherapeutics; 2019 Oct 12; 16(4):1074-1096. PubMed ID: 31432449
    [Abstract] [Full Text] [Related]


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