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Results: 1 to 20 of 114

1.

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
2.

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.

PMID:
23536093
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

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.

PMID:
12741986
[PubMed - indexed for MEDLINE]
4.

Abnormalities in the climbing fiber-Purkinje cell circuitry contribute to neuronal dysfunction in ATXN1[82Q] mice.

Barnes JA, Ebner BA, Duvick LA, Gao W, Chen G, Orr HT, Ebner TJ.

J Neurosci. 2011 Sep 7;31(36):12778-89. doi: 10.1523/JNEUROSCI.2579-11.2011.

PMID:
21900557
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

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
6.

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
7.

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]
8.

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
9.

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]
10.

Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice.

Skinner PJ, Vierra-Green CA, Emamian E, Zoghbi HY, Orr HT.

Neuromolecular Med. 2002;1(1):33-42.

PMID:
12025814
[PubMed - indexed for MEDLINE]
11.

Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice.

Zu T, Duvick LA, Kaytor MD, Berlinger MS, Zoghbi HY, Clark HB, Orr HT.

J Neurosci. 2004 Oct 6;24(40):8853-61.

PMID:
15470152
[PubMed - indexed for MEDLINE]
Free Article
12.

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
13.

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
14.

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
15.

Hsp70/Hsc70 regulates the effect phosphorylation has on stabilizing ataxin-1.

Jorgensen ND, Andresen JM, Pitt JE, Swenson MA, Zoghbi HY, Orr HT.

J Neurochem. 2007 Sep;102(6):2040-8. Epub 2007 Jun 1.

PMID:
17540008
[PubMed - indexed for MEDLINE]
16.

Suppression of calbindin-D28k expression exacerbates SCA1 phenotype in a disease mouse model.

Vig PJ, Wei J, Shao Q, Lopez ME, Halperin R, Gerber J.

Cerebellum. 2012 Sep;11(3):718-32. doi: 10.1007/s12311-011-0323-9.

PMID:
22076800
[PubMed - indexed for MEDLINE]
17.

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
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.

PMID:
23719801
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

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]
20.

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

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