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Items: 1 to 20 of 104


RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1.

Park J, Al-Ramahi I, Tan Q, Mollema N, Diaz-Garcia JR, Gallego-Flores T, Lu HC, Lagalwar S, Duvick L, Kang H, Lee Y, Jafar-Nejad P, Sayegh LS, Richman R, Liu X, Gao Y, Shaw CA, Arthur JSC, Orr HT, Westbrook TF, Botas J, Zoghbi HY.

Nature. 2013 Jun 20;498(7454):325-331. doi: 10.1038/nature12204. Epub 2013 May 29.


New hope for therapy in neurodegenerative diseases.

do Carmo Costa M, Paulson HL.

Cell Res. 2013 Oct;23(10):1159-60. doi: 10.1038/cr.2013.96. Epub 2013 Jul 23.


CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation.

Al-Ramahi I, Lam YC, Chen HK, de Gouyon B, Zhang M, Pérez AM, Branco J, de Haro M, Patterson C, Zoghbi HY, Botas J.

J Biol Chem. 2006 Sep 8;281(36):26714-24. Epub 2006 Jul 10.


Polyglutamine disease toxicity is regulated by Nemo-like kinase in spinocerebellar ataxia type 1.

Ju H, Kokubu H, Todd TW, Kahle JJ, Kim S, Richman R, Chirala K, Orr HT, Zoghbi HY, Lim J.

J Neurosci. 2013 May 29;33(22):9328-36. doi: 10.1523/JNEUROSCI.3465-12.2013.


Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.

Lim J, Crespo-Barreto J, Jafar-Nejad P, Bowman AB, Richman R, Hill DE, Orr HT, Zoghbi HY.

Nature. 2008 Apr 10;452(7188):713-8. doi: 10.1038/nature06731. Epub 2008 Mar 12.


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 2nd, Safaya E.

Cerebellum. 2011 Jun;10(2):254-66. doi: 10.1007/s12311-011-0262-5.


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.


Dopamine D2 receptor signaling modulates mutant ataxin-1 S776 phosphorylation and aggregation.

Hearst SM, Lopez ME, Shao Q, Liu Y, Vig PJ.

J Neurochem. 2010 Aug;114(3):706-16. doi: 10.1111/j.1471-4159.2010.06791.x. Epub 2010 Apr 30.


A novel function of Ataxin-1 in the modulation of PP2A activity is dysregulated in the spinocerebellar ataxia type 1.

Sánchez I, Piñol P, Corral-Juan M, Pandolfo M, Matilla-Dueñas A.

Hum Mol Genet. 2013 Sep 1;22(17):3425-37. doi: 10.1093/hmg/ddt197. Epub 2013 Apr 29.


Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis.

Crespo-Barreto J, Fryer JD, Shaw CA, Orr HT, Zoghbi HY.

PLoS Genet. 2010 Jul 8;6(7):e1001021. doi: 10.1371/journal.pgen.1001021.


SCA1-phosphorylation, a regulator of Ataxin-1 function and pathogenesis.

Orr HT.

Prog Neurobiol. 2012 Dec;99(3):179-85. doi: 10.1016/j.pneurobio.2012.04.003. Epub 2012 Apr 16. Review.


Beyond the glutamine expansion: influence of posttranslational modifications of ataxin-1 in the pathogenesis of spinocerebellar ataxia type 1.

Ju H, Kokubu H, Lim J.

Mol Neurobiol. 2014 Dec;50(3):866-874. doi: 10.1007/s12035-014-8703-z. Epub 2014 Apr 22. Review.


Identification of a novel phosphorylation site in ataxin-1.

Vierra-Green CA, Orr HT, Zoghbi HY, Ferrington DA.

Biochim Biophys Acta. 2005 May 15;1744(1):11-8. Epub 2004 Nov 10.


Systems biology analysis of Drosophila in vivo screen data elucidates core networks for DNA damage repair in SCA1.

Barclay SS, Tamura T, Ito H, Fujita K, Tagawa K, Shimamura T, Katsuta A, Shiwaku H, Sone M, Imoto S, Miyano S, Okazawa H.

Hum Mol Genet. 2014 Mar 1;23(5):1345-64. doi: 10.1093/hmg/ddt524. Epub 2013 Oct 31.


Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1.

Zoghbi HY, Orr HT.

J Biol Chem. 2009 Mar 20;284(12):7425-9. doi: 10.1074/jbc.R800041200. Epub 2008 Oct 28. Review.


Vascular endothelial growth factor ameliorates the ataxic phenotype in a mouse model of spinocerebellar ataxia type 1.

Cvetanovic M, Patel JM, Marti HH, Kini AR, Opal P.

Nat Med. 2011 Oct 16;17(11):1445-7. doi: 10.1038/nm.2494.


Structural basis of the phosphorylation dependent complex formation of neurodegenerative disease protein Ataxin-1 and RBM17.

Kim E, Lee Y, Choi S, Song JJ.

Biochem Biophys Res Commun. 2014 Jul 11;449(4):399-404. doi: 10.1016/j.bbrc.2014.05.063. Epub 2014 May 22.


SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776.

Duvick L, Barnes J, Ebner B, Agrawal S, Andresen M, Lim J, Giesler GJ, Zoghbi HY, Orr HT.

Neuron. 2010 Sep 23;67(6):929-35. doi: 10.1016/j.neuron.2010.08.022.


Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model.

Gehrking KM, Andresen JM, Duvick L, Lough J, Zoghbi HY, Orr HT.

Hum Mol Genet. 2011 Jun 1;20(11):2204-12. doi: 10.1093/hmg/ddr108. Epub 2011 Mar 22.


The histone deacetylase HDAC3 is essential for Purkinje cell function, potentially complicating the use of HDAC inhibitors in SCA1.

Venkatraman A, Hu YS, Didonna A, Cvetanovic M, Krbanjevic A, Bilesimo P, Opal P.

Hum Mol Genet. 2014 Jul 15;23(14):3733-45. doi: 10.1093/hmg/ddu081. Epub 2014 Mar 4.

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