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200 related items for PubMed ID: 11741393

  • 1. Reduction of Purkinje cell pathology in SCA1 transgenic mice by p53 deletion.
    Shahbazian MD, Orr HT, Zoghbi HY.
    Neurobiol Dis; 2001 Dec; 8(6):974-81. PubMed ID: 11741393
    [Abstract] [Full Text] [Related]

  • 2. Intranasal administration of IGF-I improves behavior and Purkinje cell pathology in SCA1 mice.
    Vig PJ, Subramony SH, D'Souza DR, Wei J, Lopez ME.
    Brain Res Bull; 2006 May 15; 69(5):573-9. PubMed ID: 16647585
    [Abstract] [Full Text] [Related]

  • 3. SUMO-1 interacts with mutant ataxin-1 and colocalizes to its aggregates in Purkinje cells of SCA1 transgenic mice.
    Kang S, Hong S.
    Arch Ital Biol; 2010 Dec 15; 148(4):351-63. PubMed ID: 21308649
    [Abstract] [Full Text] [Related]

  • 4. RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.
    Xia H, Mao Q, Eliason SL, Harper SQ, Martins IH, Orr HT, Paulson HL, Yang L, Kotin RM, Davidson BL.
    Nat Med; 2004 Aug 15; 10(8):816-20. PubMed ID: 15235598
    [Abstract] [Full Text] [Related]

  • 5. Progress in pathogenesis studies of spinocerebellar ataxia type 1.
    Cummings CJ, Orr HT, Zoghbi HY.
    Philos Trans R Soc Lond B Biol Sci; 1999 Jun 29; 354(1386):1079-81. PubMed ID: 10434309
    [Abstract] [Full Text] [Related]

  • 6. Identification of genes that modify ataxin-1-induced neurodegeneration.
    Fernandez-Funez P, Nino-Rosales ML, de Gouyon B, She WC, Luchak JM, Martinez P, Turiegano E, Benito J, Capovilla M, Skinner PJ, McCall A, Canal I, Orr HT, Zoghbi HY, Botas J.
    Nature; 2000 Nov 02; 408(6808):101-6. PubMed ID: 11081516
    [Abstract] [Full Text] [Related]

  • 7. Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice.
    Emamian ES, Kaytor MD, Duvick LA, Zu T, Tousey SK, Zoghbi HY, Clark HB, Orr HT.
    Neuron; 2003 May 08; 38(3):375-87. PubMed ID: 12741986
    [Abstract] [Full Text] [Related]

  • 8. The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1.
    Matilla A, Koshy BT, Cummings CJ, Isobe T, Orr HT, Zoghbi HY.
    Nature; 1997 Oct 30; 389(6654):974-8. PubMed ID: 9353121
    [Abstract] [Full Text] [Related]

  • 9. The ins and outs of a polyglutamine neurodegenerative disease: spinocerebellar ataxia type 1 (SCA1).
    Orr HT.
    Neurobiol Dis; 2000 Jun 30; 7(3):129-34. PubMed ID: 10860780
    [Abstract] [Full Text] [Related]

  • 10. Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes.
    Bowman AB, Lam YC, Jafar-Nejad P, Chen HK, Richman R, Samaco RC, Fryer JD, Kahle JJ, Orr HT, Zoghbi HY.
    Nat Genet; 2007 Mar 30; 39(3):373-9. PubMed ID: 17322884
    [Abstract] [Full Text] [Related]

  • 11. Glial S100B protein modulates mutant ataxin-1 aggregation and toxicity: TRTK12 peptide, a potential candidate for SCA1 therapy.
    Vig PJ, Hearst S, Shao Q, Lopez ME, Murphy HA, Safaya E.
    Cerebellum; 2011 Jun 30; 10(2):254-66. PubMed ID: 21384195
    [Abstract] [Full Text] [Related]

  • 12. Polyglutamine-expanded ataxin-7 upregulates Bax expression by activating p53 in cerebellar and inferior olivary neurons.
    Wang HL, Chou AH, Lin AC, Chen SY, Weng YH, Yeh TH.
    Exp Neurol; 2010 Aug 30; 224(2):486-94. PubMed ID: 20546728
    [Abstract] [Full Text] [Related]

  • 13. RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice.
    Serra HG, Duvick L, Zu T, Carlson K, Stevens S, Jorgensen N, Lysholm A, Burright E, Zoghbi HY, Clark HB, Andresen JM, Orr HT.
    Cell; 2006 Nov 17; 127(4):697-708. PubMed ID: 17110330
    [Abstract] [Full Text] [Related]

  • 14. USP7, a ubiquitin-specific protease, interacts with ataxin-1, the SCA1 gene product.
    Hong S, Kim SJ, Ka S, Choi I, Kang S.
    Mol Cell Neurosci; 2002 Jun 17; 20(2):298-306. PubMed ID: 12093161
    [Abstract] [Full Text] [Related]

  • 15. Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human.
    Huynh DP, Figueroa K, Hoang N, Pulst SM.
    Nat Genet; 2000 Sep 17; 26(1):44-50. PubMed ID: 10973246
    [Abstract] [Full Text] [Related]

  • 16. Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice.
    Skinner PJ, Vierra-Green CA, Emamian E, Zoghbi HY, Orr HT.
    Neuromolecular Med; 2002 Sep 17; 1(1):33-42. PubMed ID: 12025814
    [Abstract] [Full Text] [Related]

  • 17. 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 17; 73(2):115-21. PubMed ID: 22426494
    [Abstract] [Full Text] [Related]

  • 18. Delayed spinocerebellar ataxia in transgenic mice expressing mutant ubiquitin.
    Tsirigotis M, Tang MY, Beyers M, Zhang M, Woulfe J, Gray DA.
    Neuropathol Appl Neurobiol; 2006 Feb 17; 32(1):26-39. PubMed ID: 16409551
    [Abstract] [Full Text] [Related]

  • 19. Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice.
    Zu T, Duvick LA, Kaytor MD, Berlinger MS, Zoghbi HY, Clark HB, Orr HT.
    J Neurosci; 2004 Oct 06; 24(40):8853-61. PubMed ID: 15470152
    [Abstract] [Full Text] [Related]

  • 20. Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.
    Chen HK, Fernandez-Funez P, Acevedo SF, Lam YC, Kaytor MD, Fernandez MH, Aitken A, Skoulakis EM, Orr HT, Botas J, Zoghbi HY.
    Cell; 2003 May 16; 113(4):457-68. PubMed ID: 12757707
    [Abstract] [Full Text] [Related]

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