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Results: 1 to 20 of 134

1.

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.

PMID:
17322884
[PubMed - indexed for MEDLINE]
2.

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.

PMID:
18337722
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology.

Lam YC, Bowman AB, Jafar-Nejad P, Lim J, Richman R, Fryer JD, Hyun ED, Duvick LA, Orr HT, Botas J, Zoghbi HY.

Cell. 2006 Dec 29;127(7):1335-47.

PMID:
17190598
[PubMed - indexed for MEDLINE]
Free Article
4.

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.

PMID:
20628574
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

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.

Science. 2011 Nov 4;334(6056):690-3. doi: 10.1126/science.1212673.

PMID:
22053053
[PubMed - indexed for MEDLINE]
Free PMC Article
6.

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.

PMID:
10434309
[PubMed - indexed for MEDLINE]
Free PMC Article
7.

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.

PMID:
21427130
[PubMed - indexed for MEDLINE]
Free PMC Article
8.

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.

PMID:
23719801
[PubMed - indexed for MEDLINE]
Free PMC Article
9.

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.

PMID:
22531670
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

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.

PMID:
23630944
[PubMed - indexed for MEDLINE]
Free Article
11.

Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua.

Kim E, Lu HC, Zoghbi HY, Song JJ.

Genes Dev. 2013 Mar 15;27(6):590-5. doi: 10.1101/gad.212068.112.

PMID:
23512657
[PubMed - indexed for MEDLINE]
Free PMC Article
12.

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.

PMID:
24594842
[PubMed - indexed for MEDLINE]
13.

Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model.

Watase K, Gatchel JR, Sun Y, Emamian E, Atkinson R, Richman R, Mizusawa H, Orr HT, Shaw C, Zoghbi HY.

PLoS Med. 2007 May;4(5):e182.

PMID:
17535104
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

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.

PMID:
21308649
[PubMed - indexed for MEDLINE]
15.

Spinocerebellar ataxia type 1--modeling the pathogenesis of a polyglutamine neurodegenerative disorder in transgenic mice.

Clark HB, Orr HT.

J Neuropathol Exp Neurol. 2000 Apr;59(4):265-70. Review.

PMID:
10759181
[PubMed - indexed for MEDLINE]
16.

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.

PMID:
20869591
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

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.

PMID:
18758459
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

Phosphorylation of ATXN1 at Ser776 in the cerebellum.

Jorgensen ND, Andresen JM, Lagalwar S, Armstrong B, Stevens S, Byam CE, Duvick LA, Lai S, Jafar-Nejad P, Zoghbi HY, Clark HB, Orr HT.

J Neurochem. 2009 Jul;110(2):675-86. doi: 10.1111/j.1471-4159.2009.06164.x. Epub 2009 May 15.

PMID:
19500214
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

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.

PMID:
21384195
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

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.

PMID:
24858692
[PubMed - indexed for MEDLINE]
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