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Items: 1 to 20 of 156

1.

Schwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3.

Suga N, Katsuno M, Koike H, Banno H, Suzuki K, Hashizume A, Mano T, Iijima M, Kawagashira Y, Hirayama M, Nakamura T, Watanabe H, Tanaka F, Sobue G.

Neuropathol Appl Neurobiol. 2014 Aug;40(5):628-39. doi: 10.1111/nan.12055.

PMID:
23617879
2.

Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions.

Rüb U, de Vos RA, Brunt ER, Sebestény T, Schöls L, Auburger G, Bohl J, Ghebremedhin E, Gierga K, Seidel K, den Dunnen W, Heinsen H, Paulson H, Deller T.

Brain Pathol. 2006 Jul;16(3):218-27.

PMID:
16911479
3.

Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: removal of the CAG containing exon.

Evers MM, Tran HD, Zalachoras I, Pepers BA, Meijer OC, den Dunnen JT, van Ommen GJ, Aartsma-Rus A, van Roon-Mom WM.

Neurobiol Dis. 2013 Oct;58:49-56. doi: 10.1016/j.nbd.2013.04.019. Epub 2013 May 6.

4.
5.

Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).

Hübener J, Weber JJ, Richter C, Honold L, Weiss A, Murad F, Breuer P, Wüllner U, Bellstedt P, Paquet-Durand F, Takano J, Saido TC, Riess O, Nguyen HP.

Hum Mol Genet. 2013 Feb 1;22(3):508-18. doi: 10.1093/hmg/dds449. Epub 2012 Oct 24.

PMID:
23100324
6.

A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD.

Switonski PM, Szlachcic WJ, Krzyzosiak WJ, Figiel M.

Neurobiol Dis. 2015 Jan;73:174-88. doi: 10.1016/j.nbd.2014.09.020. Epub 2014 Oct 7.

7.

Polyglutamine-expanded ataxin-3 impairs long-term depression in Purkinje neurons of SCA3 transgenic mouse by inhibiting HAT and impairing histone acetylation.

Chou AH, Chen YL, Hu SH, Chang YM, Wang HL.

Brain Res. 2014 Oct 2;1583:220-9. doi: 10.1016/j.brainres.2014.08.019. Epub 2014 Aug 17.

PMID:
25139423
8.

Machado-Joseph disease/spinocerebellar ataxia type 3.

Paulson H.

Handb Clin Neurol. 2012;103:437-49. doi: 10.1016/B978-0-444-51892-7.00027-9. Review.

9.

Inflammatory genes are upregulated in expanded ataxin-3-expressing cell lines and spinocerebellar ataxia type 3 brains.

Evert BO, Vogt IR, Kindermann C, Ozimek L, de Vos RA, Brunt ER, Schmitt I, Klockgether T, Wüllner U.

J Neurosci. 2001 Aug 1;21(15):5389-96.

10.

[Polyglutamine-expanded ataxin-3 is degraded by autophagy].

Xiao H, Tang J, Hu Z, Tan J, Tang B, Jiang Z.

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2010 Feb;27(1):23-8. doi: 10.3760/cma.j.issn.1003-9406.2010.01.005. Chinese.

PMID:
20140862
11.

Neuronal intranuclear inclusions, dysregulation of cytokine expression and cell death in spinocerebellar ataxia type 3.

Evert BO, Schelhaas J, Fleischer H, de Vos RA, Brunt ER, Stenzel W, Klockgether T, Wüllner U.

Clin Neuropathol. 2006 Nov-Dec;25(6):272-81.

PMID:
17140157
12.
13.

Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3.

Evert BO, Vogt IR, Vieira-Saecker AM, Ozimek L, de Vos RA, Brunt ER, Klockgether T, Wüllner U.

J Neuropathol Exp Neurol. 2003 Oct;62(10):1006-18.

PMID:
14575237
14.

Intermediate CAG repeat lengths (53,54) for MJD/SCA3 are associated with an abnormal phenotype.

van Alfen N, Sinke RJ, Zwarts MJ, Gabreëls-Festen A, Praamstra P, Kremer BP, Horstink MW.

Ann Neurol. 2001 Jun;49(6):805-7.

PMID:
11409435
15.

Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence.

Bichelmeier U, Schmidt T, Hübener J, Boy J, Rüttiger L, Häbig K, Poths S, Bonin M, Knipper M, Schmidt WJ, Wilbertz J, Wolburg H, Laccone F, Riess O.

J Neurosci. 2007 Jul 11;27(28):7418-28.

16.

In vivo assessment of riluzole as a potential therapeutic drug for spinocerebellar ataxia type 3.

Schmidt J, Schmidt T, Golla M, Lehmann L, Weber JJ, Hübener-Schmid J, Riess O.

J Neurochem. 2016 Jul;138(1):150-62. doi: 10.1111/jnc.13606.

17.

Potassium channel dysfunction and depolarized resting membrane potential in a cell model of SCA3.

Jeub M, Herbst M, Spauschus A, Fleischer H, Klockgether T, Wuellner U, Evert BO.

Exp Neurol. 2006 Sep;201(1):182-92. Epub 2006 Jun 12.

PMID:
16765348
18.

Caring for Machado-Joseph disease: current understanding and how to help patients.

D'Abreu A, França MC Jr, Paulson HL, Lopes-Cendes I.

Parkinsonism Relat Disord. 2010 Jan;16(1):2-7. doi: 10.1016/j.parkreldis.2009.08.012. Epub 2009 Oct 6. Review.

19.

CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegeneration.

Liman J, Deeg S, Voigt A, Voßfeldt H, Dohm CP, Karch A, Weishaupt J, Schulz JB, Bähr M, Kermer P.

J Neurochem. 2014 Jun;129(6):1013-23. doi: 10.1111/jnc.12684. Epub 2014 Mar 24.

20.

Inactivation of PNKP by mutant ATXN3 triggers apoptosis by activating the DNA damage-response pathway in SCA3.

Gao R, Liu Y, Silva-Fernandes A, Fang X, Paulucci-Holthauzen A, Chatterjee A, Zhang HL, Matsuura T, Choudhary S, Ashizawa T, Koeppen AH, Maciel P, Hazra TK, Sarkar PS.

PLoS Genet. 2015 Jan 15;11(1):e1004834. doi: 10.1371/journal.pgen.1004834. eCollection 2015 Jan.

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