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387 related items for PubMed ID: 20510362

  • 1. Motor uncoordination and neuropathology in a transgenic mouse model of Machado-Joseph disease lacking intranuclear inclusions and ataxin-3 cleavage products.
    Silva-Fernandes A, Costa Mdo C, Duarte-Silva S, Oliveira P, Botelho CM, Martins L, Mariz JA, Ferreira T, Ribeiro F, Correia-Neves M, Costa C, Maciel P.
    Neurobiol Dis; 2010 Oct; 40(1):163-76. PubMed ID: 20510362
    [Abstract] [Full Text] [Related]

  • 2. 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; 73():174-88. PubMed ID: 25301414
    [Abstract] [Full Text] [Related]

  • 3. Caffeine and adenosine A(2A) receptor inactivation decrease striatal neuropathology in a lentiviral-based model of Machado-Joseph disease.
    Gonçalves N, Simões AT, Cunha RA, de Almeida LP.
    Ann Neurol; 2013 May; 73(5):655-66. PubMed ID: 23625556
    [Abstract] [Full Text] [Related]

  • 4. Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice.
    Nóbrega C, Nascimento-Ferreira I, Onofre I, Albuquerque D, Hirai H, Déglon N, de Almeida LP.
    PLoS One; 2013 May; 8(1):e52396. PubMed ID: 23349684
    [Abstract] [Full Text] [Related]

  • 5. Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease.
    Alves S, Régulier E, Nascimento-Ferreira I, Hassig R, Dufour N, Koeppen A, Carvalho AL, Simões S, de Lima MC, Brouillet E, Gould VC, Déglon N, de Almeida LP.
    Hum Mol Genet; 2008 Jul 15; 17(14):2071-83. PubMed ID: 18385100
    [Abstract] [Full Text] [Related]

  • 6. Involvement of Onuf's nucleus in Machado-Joseph disease: a morphometric and immunohistochemical study.
    Shimizu H, Yamada M, Toyoshima Y, Ikeuchi T, Onodera O, Takahashi H.
    Acta Neuropathol; 2010 Oct 15; 120(4):439-48. PubMed ID: 20503052
    [Abstract] [Full Text] [Related]

  • 7. A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration.
    Goti D, Katzen SM, Mez J, Kurtis N, Kiluk J, Ben-Haïem L, Jenkins NA, Copeland NG, Kakizuka A, Sharp AH, Ross CA, Mouton PR, Colomer V.
    J Neurosci; 2004 Nov 10; 24(45):10266-79. PubMed ID: 15537899
    [Abstract] [Full Text] [Related]

  • 8. A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats.
    Boy J, Schmidt T, Schumann U, Grasshoff U, Unser S, Holzmann C, Schmitt I, Karl T, Laccone F, Wolburg H, Ibrahim S, Riess O.
    Neurobiol Dis; 2010 Feb 10; 37(2):284-93. PubMed ID: 19699305
    [Abstract] [Full Text] [Related]

  • 9. A mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice.
    Colomer Gould VF, Goti D, Pearce D, Gonzalez GA, Gao H, Bermudez de Leon M, Jenkins NA, Copeland NG, Ross CA, Brown DR.
    Neurobiol Dis; 2007 Sep 10; 27(3):362-9. PubMed ID: 17632007
    [Abstract] [Full Text] [Related]

  • 10. Polyglutamine diseases: the special case of ataxin-3 and Machado-Joseph disease.
    Matos CA, de Macedo-Ribeiro S, Carvalho AL.
    Prog Neurobiol; 2011 Sep 15; 95(1):26-48. PubMed ID: 21740957
    [Abstract] [Full Text] [Related]

  • 11. 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 15; 134(Pt 5):1400-15. PubMed ID: 21478185
    [Abstract] [Full Text] [Related]

  • 12. YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit.
    Cemal CK, Carroll CJ, Lawrence L, Lowrie MB, Ruddle P, Al-Mahdawi S, King RH, Pook MA, Huxley C, Chamberlain S.
    Hum Mol Genet; 2002 May 01; 11(9):1075-94. PubMed ID: 11978767
    [Abstract] [Full Text] [Related]

  • 13. Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: removal of the CAG containing exon.
    Evers MM, Tran HD, Zalachoras I, Pepers BA, Meijer OC, den Dunnen JT, van Ommen GJ, Aartsma-Rus A, van Roon-Mom WM.
    Neurobiol Dis; 2013 Oct 01; 58():49-56. PubMed ID: 23659897
    [Abstract] [Full Text] [Related]

  • 14. Compromised mitochondrial complex II in models of Machado-Joseph disease.
    Laço MN, Oliveira CR, Paulson HL, Rego AC.
    Biochim Biophys Acta; 2012 Feb 01; 1822(2):139-49. PubMed ID: 22037589
    [Abstract] [Full Text] [Related]

  • 15. Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease.
    Silva-Fernandes A, Duarte-Silva S, Neves-Carvalho A, Amorim M, Soares-Cunha C, Oliveira P, Thirstrup K, Teixeira-Castro A, Maciel P.
    Neurotherapeutics; 2014 Apr 01; 11(2):433-49. PubMed ID: 24477711
    [Abstract] [Full Text] [Related]

  • 16. Polyglutamine-expanded ataxin-3 impairs long-term depression in Purkinje neurons of SCA3 transgenic mouse by inhibiting HAT and impairing histone acetylation.
    Chou AH, Chen YL, Hu SH, Chang YM, Wang HL.
    Brain Res; 2014 Oct 02; 1583():220-9. PubMed ID: 25139423
    [Abstract] [Full Text] [Related]

  • 17. Defining a metabolic phenotype in the brain of a transgenic mouse model of spinocerebellar ataxia 3.
    Griffin JL, Cemal CK, Pook MA.
    Physiol Genomics; 2004 Feb 13; 16(3):334-40. PubMed ID: 14679302
    [Abstract] [Full Text] [Related]

  • 18. [Polyglutamine-expanded ataxin-3 is degraded by autophagy].
    Xiao H, Tang J, Hu Z, Tan J, Tang B, Jiang Z.
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi; 2010 Feb 13; 27(1):23-8. PubMed ID: 20140862
    [Abstract] [Full Text] [Related]

  • 19. Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions.
    Rüb U, de Vos RA, Brunt ER, Sebestény T, Schöls L, Auburger G, Bohl J, Ghebremedhin E, Gierga K, Seidel K, den Dunnen W, Heinsen H, Paulson H, Deller T.
    Brain Pathol; 2006 Jul 13; 16(3):218-27. PubMed ID: 16911479
    [Abstract] [Full Text] [Related]

  • 20. Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence.
    Bichelmeier U, Schmidt T, Hübener J, Boy J, Rüttiger L, Häbig K, Poths S, Bonin M, Knipper M, Schmidt WJ, Wilbertz J, Wolburg H, Laccone F, Riess O.
    J Neurosci; 2007 Jul 11; 27(28):7418-28. PubMed ID: 17626202
    [Abstract] [Full Text] [Related]

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