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Items: 1 to 20 of 246

1.

Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.

Garden GA, Libby RT, Fu YH, Kinoshita Y, Huang J, Possin DE, Smith AC, Martinez RA, Fine GC, Grote SK, Ware CB, Einum DD, Morrison RS, Ptacek LJ, Sopher BL, La Spada AR.

J Neurosci. 2002 Jun 15;22(12):4897-905.

2.

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.

3.

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

Klement IA, Skinner PJ, Kaytor MD, Yi H, Hersch SM, Clark HB, Zoghbi HY, Orr HT.

Cell. 1998 Oct 2;95(1):41-53.

4.
5.

Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice.

Yvert G, Lindenberg KS, Picaud S, Landwehrmeyer GB, Sahel JA, Mandel JL.

Hum Mol Genet. 2000 Oct 12;9(17):2491-506.

PMID:
11030754
6.

SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types.

Yvert G, Lindenberg KS, Devys D, Helmlinger D, Landwehrmeyer GB, Mandel JL.

Hum Mol Genet. 2001 Aug 1;10(16):1679-92.

PMID:
11487572
7.

Relationship between ataxin-1 nuclear inclusions and Purkinje cell specific proteins in SCA-1 transgenic mice.

Vig PJ, Subramony SH, Qin Z, McDaniel DO, Fratkin JD.

J Neurol Sci. 2000 Mar 15;174(2):100-10.

PMID:
10727695
8.

Spinocerebellar ataxia type 7 cerebellar disease requires the coordinated action of mutant ataxin-7 in neurons and glia, and displays non-cell-autonomous bergmann glia degeneration.

Furrer SA, Mohanachandran MS, Waldherr SM, Chang C, Damian VA, Sopher BL, Garden GA, La Spada AR.

J Neurosci. 2011 Nov 9;31(45):16269-78. doi: 10.1523/JNEUROSCI.4000-11.2011.

9.
10.

Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model.

Helmlinger D, Abou-Sleymane G, Yvert G, Rousseau S, Weber C, Trottier Y, Mandel JL, Devys D.

J Neurosci. 2004 Feb 25;24(8):1881-7.

11.

Polyglutamine-expanded ataxin-7 upregulates Bax expression by activating p53 in cerebellar and inferior olivary neurons.

Wang HL, Chou AH, Lin AC, Chen SY, Weng YH, Yeh TH.

Exp Neurol. 2010 Aug;224(2):486-94. doi: 10.1016/j.expneurol.2010.05.011. Epub 2010 May 28.

PMID:
20546728
12.

Hsp70 and Hsp40 chaperones do not modulate retinal phenotype in SCA7 mice.

Helmlinger D, Bonnet J, Mandel JL, Trottier Y, Devys D.

J Biol Chem. 2004 Dec 31;279(53):55969-77. Epub 2004 Oct 19.

13.

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.

14.

Ataxin-7 expression analysis in controls and spinocerebellar ataxia type 7 patients.

Einum DD, Townsend JJ, Ptácek LJ, Fu YH.

Neurogenetics. 2001 Mar;3(2):83-90.

PMID:
11354830
15.

Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport.

Custer SK, Garden GA, Gill N, Rueb U, Libby RT, Schultz C, Guyenet SJ, Deller T, Westrum LE, Sopher BL, La Spada AR.

Nat Neurosci. 2006 Oct;9(10):1302-11. Epub 2006 Aug 27.

PMID:
16936724
16.

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.

17.

Polyglutamine-expanded ataxin-7 causes cerebellar dysfunction by inducing transcriptional dysregulation.

Chou AH, Chen CY, Chen SY, Chen WJ, Chen YL, Weng YS, Wang HL.

Neurochem Int. 2010 Jan;56(2):329-39. doi: 10.1016/j.neuint.2009.11.003. Epub 2009 Nov 10.

PMID:
19909779
18.

Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7.

Furrer SA, Waldherr SM, Mohanachandran MS, Baughn TD, Nguyen KT, Sopher BL, Damian VA, Garden GA, La Spada AR.

Hum Mol Genet. 2013 Mar 1;22(5):890-903. doi: 10.1093/hmg/dds495. Epub 2012 Nov 29.

19.

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
20.

Expression of ataxin-7 in CNS and non-CNS tissue of normal and SCA7 individuals.

Jonasson J, Ström AL, Hart P, Brännström T, Forsgren L, Holmberg M.

Acta Neuropathol. 2002 Jul;104(1):29-37. Epub 2002 Mar 2.

PMID:
12070661

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