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278 related items for PubMed ID: 24859968

  • 1. The autophagy/lysosome pathway is impaired in SCA7 patients and SCA7 knock-in mice.
    Alves S, Cormier-Dequaire F, Marinello M, Marais T, Muriel MP, Beaumatin F, Charbonnier-Beaupel F, Tahiri K, Seilhean D, El Hachimi K, Ruberg M, Stevanin G, Barkats M, den Dunnen W, Priault M, Brice A, Durr A, Corvol JC, Sittler A.
    Acta Neuropathol; 2014 Nov; 128(5):705-22. PubMed ID: 24859968
    [Abstract] [Full Text] [Related]

  • 2. Autophagy: polyQ toxic fragment turnover.
    Duncan C, Papanikolaou T, Ellerby LM.
    Autophagy; 2010 Feb; 6(2):312-4. PubMed ID: 20104018
    [Abstract] [Full Text] [Related]

  • 3. Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease.
    Nascimento-Ferreira I, Santos-Ferreira T, Sousa-Ferreira L, Auregan G, Onofre I, Alves S, Dufour N, Colomer Gould VF, Koeppen A, Déglon N, Pereira de Almeida L.
    Brain; 2011 May; 134(Pt 5):1400-15. PubMed ID: 21478185
    [Abstract] [Full Text] [Related]

  • 4. 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]

  • 5. 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]

  • 6. 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 01; 136(Pt 6):1732-45. PubMed ID: 23518714
    [Abstract] [Full Text] [Related]

  • 7. 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]

  • 8. Similarities between spinocerebellar ataxia type 7 (SCA7) cell models and human brain: proteins recruited in inclusions and activation of caspase-3.
    Zander C, Takahashi J, El Hachimi KH, Fujigasaki H, Albanese V, Lebre AS, Stevanin G, Duyckaerts C, Brice A.
    Hum Mol Genet; 2001 Oct 15; 10(22):2569-79. PubMed ID: 11709544
    [Abstract] [Full Text] [Related]

  • 9. SUMOylation by SUMO2 is implicated in the degradation of misfolded ataxin-7 via RNF4 in SCA7 models.
    Marinello M, Werner A, Giannone M, Tahiri K, Alves S, Tesson C, den Dunnen W, Seeler JS, Brice A, Sittler A.
    Dis Model Mech; 2019 Jan 11; 12(1):. PubMed ID: 30559154
    [Abstract] [Full Text] [Related]

  • 10. Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration.
    Garden GA, La Spada AR.
    Cerebellum; 2008 Jan 11; 7(2):138-49. PubMed ID: 18418675
    [Abstract] [Full Text] [Related]

  • 11. 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]

  • 12. 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]

  • 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. SUMOylation attenuates the aggregation propensity and cellular toxicity of the polyglutamine expanded ataxin-7.
    Janer A, Werner A, Takahashi-Fujigasaki J, Daret A, Fujigasaki H, Takada K, Duyckaerts C, Brice A, Dejean A, Sittler A.
    Hum Mol Genet; 2010 Jan 01; 19(1):181-95. PubMed ID: 19843541
    [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. 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]

  • 17. Differential degradation of full-length and cleaved ataxin-7 fragments in a novel stable inducible SCA7 model.
    Yu X, Ajayi A, Boga NR, Ström AL.
    J Mol Neurosci; 2012 Jun 28; 47(2):219-33. PubMed ID: 22367614
    [Abstract] [Full Text] [Related]

  • 18. Ataxin-7 expression analysis in controls and spinocerebellar ataxia type 7 patients.
    Einum DD, Townsend JJ, Ptácek LJ, Fu YH.
    Neurogenetics; 2001 Mar 28; 3(2):83-90. PubMed ID: 11354830
    [Abstract] [Full Text] [Related]

  • 19. A novel central nervous system-enriched spinocerebellar ataxia type 7 gene product.
    Einum DD, Clark AM, Townsend JJ, Ptacek LJ, Fu YH.
    Arch Neurol; 2003 Jan 28; 60(1):97-103. PubMed ID: 12533095
    [Abstract] [Full Text] [Related]

  • 20. Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7.
    La Spada AR, Fu YH, Sopher BL, Libby RT, Wang X, Li LY, Einum DD, Huang J, Possin DE, Smith AC, Martinez RA, Koszdin KL, Treuting PM, Ware CB, Hurley JB, Ptácek LJ, Chen S.
    Neuron; 2001 Sep 27; 31(6):913-27. PubMed ID: 11580893
    [Abstract] [Full Text] [Related]

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