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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.


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.


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.


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 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.


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.


Cell biology of spinocerebellar ataxia.

Orr HT.

J Cell Biol. 2012 Apr 16;197(2):167-77. doi: 10.1083/jcb.201105092. Review.


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.


Toward cell specificity in SCA1.

Humbert S, Saudou F.

Neuron. 2002 May 30;34(5):669-70. 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.


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.


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.


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.


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.


Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1.

Lin X, Antalffy B, Kang D, Orr HT, Zoghbi HY.

Nat Neurosci. 2000 Feb;3(2):157-63.


Neurodegeneration: a question of balance.

Thompson LM.

Nature. 2008 Apr 10;452(7188):707-8. doi: 10.1038/452707a. No abstract available.


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.


Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration.

Jafar-Nejad P, Ward CS, Richman R, Orr HT, Zoghbi HY.

Proc Natl Acad Sci U S A. 2011 Feb 1;108(5):2142-7. doi: 10.1073/pnas.1018748108. Epub 2011 Jan 18.


Molecular pathogenesis of spinocerebellar ataxia type 1 disease.

Kang S, Hong S.

Mol Cells. 2009 Jun 30;27(6):621-7. doi: 10.1007/s10059-009-0095-y. Epub 2009 Jun 22. Review.


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.

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