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Similar articles for PubMed (Select 18758459)


miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis.

Lee Y, Samaco RC, Gatchel JR, Thaller C, Orr HT, Zoghbi HY.

Nat Neurosci. 2008 Oct;11(10):1137-9. doi: 10.1038/nn.2183. Epub 2008 Aug 31.


Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum.

Ebner BA, Ingram MA, Barnes JA, Duvick LA, Frisch JL, Clark HB, Zoghbi HY, Ebner TJ, Orr HT.

J Neurosci. 2013 Mar 27;33(13):5806-20. doi: 10.1523/JNEUROSCI.6311-11.2013.


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.


Altered Purkinje cell miRNA expression and SCA1 pathogenesis.

Rodriguez-Lebron E, Liu G, Keiser M, Behlke MA, Davidson BL.

Neurobiol Dis. 2013 Jun;54:456-63. doi: 10.1016/j.nbd.2013.01.019. Epub 2013 Jan 30.


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;39(3):373-9. Epub 2007 Feb 18.


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.


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 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.


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.


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.


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.


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.


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.


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;148(4):351-63. doi: 10.4449/aib.v148i4.1201.


Transcriptional dysregulation of TrkA associates with neurodegeneration in spinocerebellar ataxia type 17.

Shah AG, Friedman MJ, Huang S, Roberts M, Li XJ, Li S.

Hum Mol Genet. 2009 Nov 1;18(21):4141-52. doi: 10.1093/hmg/ddp363. Epub 2009 Jul 30.


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. Review.


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 8;38(3):375-87.


Focused cerebellar laser light induced hyperthermia improves symptoms and pathology of polyglutamine disease SCA1 in a mouse model.

Hearst SM, Shao Q, Lopez M, Raucher D, Vig PJ.

Cerebellum. 2014 Oct;13(5):596-606. doi: 10.1007/s12311-014-0576-1.


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;20(2):298-306.

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