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Protein interacting with C kinase (PICK1) is a suppressor of spinocerebellar ataxia 3-associated neurodegeneration in Drosophila.

McGurk L, Bonini NM.

Hum Mol Genet. 2012 Jan 1;21(1):76-84. doi: 10.1093/hmg/ddr439. Epub 2011 Sep 23.


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.


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.


RNA toxicity is a component of ataxin-3 degeneration in Drosophila.

Li LB, Yu Z, Teng X, Bonini NM.

Nature. 2008 Jun 19;453(7198):1107-11. doi: 10.1038/nature06909. Epub 2008 Apr 30.


Lithium chloride alleviates neurodegeneration partly by inhibiting activity of GSK3β in a SCA3 Drosophila model.

Jia DD, Zhang L, Chen Z, Wang CR, Huang FZ, Duan RH, Xia K, Tang BS, Jiang H.

Cerebellum. 2013 Dec;12(6):892-901. doi: 10.1007/s12311-013-0498-3.


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.


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.


Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3.

Jung J, Xu K, Lessing D, Bonini NM.

Hum Mol Genet. 2009 Dec 15;18(24):4843-52. doi: 10.1093/hmg/ddp456. Epub 2009 Sep 25.


Polyglutamine diseases: the special case of ataxin-3 and Machado-Joseph disease.

Matos CA, de Macedo-Ribeiro S, Carvalho AL.

Prog Neurobiol. 2011 Sep 15;95(1):26-48. doi: 10.1016/j.pneurobio.2011.06.007. Epub 2011 Jun 28. Review.


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.


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.


[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.


CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3.

Mueller T, Breuer P, Schmitt I, Walter J, Evert BO, Wüllner U.

Hum Mol Genet. 2009 Sep 1;18(17):3334-43. doi: 10.1093/hmg/ddp274. Epub 2009 Jun 19.


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.


MicroRNA pathways modulate polyglutamine-induced neurodegeneration.

Bilen J, Liu N, Burnett BG, Pittman RN, Bonini NM.

Mol Cell. 2006 Oct 6;24(1):157-63.


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.


In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis.

Williams AJ, Knutson TM, Colomer Gould VF, Paulson HL.

Neurobiol Dis. 2009 Mar;33(3):342-53. doi: 10.1016/j.nbd.2008.10.016. Epub 2008 Nov 8. Erratum in: Neurobiol Dis. 2012 May;46(2):503.


Flanking domain stability modulates the aggregation kinetics of a polyglutamine disease protein.

Saunders HM, Gilis D, Rooman M, Dehouck Y, Robertson AL, Bottomley SP.

Protein Sci. 2011 Oct;20(10):1675-81. doi: 10.1002/pro.698. Epub 2011 Aug 18.

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