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Glutamine repeats and neurodegeneration.
Zoghbi HY, Orr HT. Zoghbi HY, et al. Among authors: orr ht. Annu Rev Neurosci. 2000;23:217-47. doi: 10.1146/annurev.neuro.23.1.217. Annu Rev Neurosci. 2000. PMID: 10845064 Review.
Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1.
Cvetanovic M, Ingram M, Orr H, Opal P. Cvetanovic M, et al. Among authors: orr h. Neuroscience. 2015 Mar 19;289:289-99. doi: 10.1016/j.neuroscience.2015.01.003. Epub 2015 Jan 14. Neuroscience. 2015. PMID: 25595967 Free PMC article.
The ins and outs of a polyglutamine neurodegenerative disease: spinocerebellar ataxia type 1 (SCA1).
Orr HT. Orr HT. Neurobiol Dis. 2000 Jun;7(3):129-34. doi: 10.1006/nbdi.2000.0305. Neurobiol Dis. 2000. PMID: 10860780 Review.
SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat.
Burright EN, Clark HB, Servadio A, Matilla T, Feddersen RM, Yunis WS, Duvick LA, Zoghbi HY, Orr HT. Burright EN, et al. Among authors: orr ht. Cell. 1995 Sep 22;82(6):937-48. doi: 10.1016/0092-8674(95)90273-2. Cell. 1995. PMID: 7553854
The unstable repeats--three evolving faces of neurological disease.
Nelson DL, Orr HT, Warren ST. Nelson DL, et al. Among authors: orr ht. Neuron. 2013 Mar 6;77(5):825-43. doi: 10.1016/j.neuron.2013.02.022. Neuron. 2013. PMID: 23473314 Free PMC article. Review.
Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua.
Fryer JD, Yu P, Kang H, Mandel-Brehm C, Carter AN, Crespo-Barreto J, Gao Y, Flora A, Shaw C, Orr HT, Zoghbi HY. Fryer JD, et al. Among authors: orr ht. Science. 2011 Nov 4;334(6056):690-3. doi: 10.1126/science.1212673. Science. 2011. PMID: 22053053 Free PMC article.
PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1.
Bondar VV, Adamski CJ, Onur TS, Tan Q, Wang L, Diaz-Garcia J, Park J, Orr HT, Botas J, Zoghbi HY. Bondar VV, et al. Among authors: orr ht. Hum Mol Genet. 2018 Aug 15;27(16):2863-2873. doi: 10.1093/hmg/ddy200. Hum Mol Genet. 2018. PMID: 29860311 Free PMC article.
A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1.
Lasagna-Reeves CA, Rousseaux MW, Guerrero-Muñoz MJ, Park J, Jafar-Nejad P, Richman R, Lu N, Sengupta U, Litvinchuk A, Orr HT, Kayed R, Zoghbi HY. Lasagna-Reeves CA, et al. Among authors: orr ht. Elife. 2015 May 19;4:e07558. doi: 10.7554/eLife.07558. Elife. 2015. PMID: 25988806 Free PMC article.
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. Jafar-Nejad P, et al. Among authors: orr ht. Proc Natl Acad Sci U S A. 2011 Feb 1;108(5):2142-7. doi: 10.1073/pnas.1018748108. Epub 2011 Jan 18. Proc Natl Acad Sci U S A. 2011. PMID: 21245341 Free PMC article.
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. Crespo-Barreto J, et al. Among authors: orr ht. PLoS Genet. 2010 Jul 8;6(7):e1001021. doi: 10.1371/journal.pgen.1001021. PLoS Genet. 2010. PMID: 20628574 Free PMC article.
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