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FOXO4-dependent upregulation of superoxide dismutase-2 in response to oxidative stress is impaired in spinocerebellar ataxia type 3.

Araujo J, Breuer P, Dieringer S, Krauss S, Dorn S, Zimmermann K, Pfeifer A, Klockgether T, Wuellner U, Evert BO.

Hum Mol Genet. 2011 Aug 1;20(15):2928-41. doi: 10.1093/hmg/ddr197. Epub 2011 May 2.


Aqueous extract of Glycyrrhiza inflata inhibits aggregation by upregulating PPARGC1A and NFE2L2-ARE pathways in cell models of spinocerebellar ataxia 3.

Chen CM, Weng YT, Chen WL, Lin TH, Chao CY, Lin CH, Chen IC, Lee LC, Lin HY, Wu YR, Chen YC, Chang KH, Tang HY, Cheng ML, Lee-Chen GJ, Lin JY.

Free Radic Biol Med. 2014 Jun;71:339-50. doi: 10.1016/j.freeradbiomed.2014.03.023. Epub 2014 Mar 25.


Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice.

Rodríguez-Lebrón E, Costa Mdo C, Luna-Cancalon K, Peron TM, Fischer S, Boudreau RL, Davidson BL, Paulson HL.

Mol Ther. 2013 Oct;21(10):1909-18. doi: 10.1038/mt.2013.152. Epub 2013 Jul 3. Erratum in: Mol Ther. 2014 Apr;22(4):891. Costa, Maria doCarmo [corrected to Costa, Maria do Carmo].


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.


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.


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.


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.


Decreased antioxidant enzyme activity and increased mitochondrial DNA damage in cellular models of Machado-Joseph disease.

Yu YC, Kuo CL, Cheng WL, Liu CS, Hsieh M.

J Neurosci Res. 2009 Jun;87(8):1884-91. doi: 10.1002/jnr.22011.


miR-25 alleviates polyQ-mediated cytotoxicity by silencing ATXN3.

Huang F, Zhang L, Long Z, Chen Z, Hou X, Wang C, Peng H, Wang J, Li J, Duan R, Xia K, Chuang DM, Tang B, Jiang H.

FEBS Lett. 2014 Dec 20;588(24):4791-8. doi: 10.1016/j.febslet.2014.11.013. Epub 2014 Nov 20.


The role of the mammalian DNA end-processing enzyme polynucleotide kinase 3'-phosphatase in spinocerebellar ataxia type 3 pathogenesis.

Chatterjee A, Saha S, Chakraborty A, Silva-Fernandes A, Mandal SM, Neves-Carvalho A, Liu Y, Pandita RK, Hegde ML, Hegde PM, Boldogh I, Ashizawa T, Koeppen AH, Pandita TK, Maciel P, Sarkar PS, Hazra TK.

PLoS Genet. 2015 Jan 29;11(1):e1004749. doi: 10.1371/journal.pgen.1004749. eCollection 2015 Jan.


Absence of ataxin-3 leads to cytoskeletal disorganization and increased cell death.

Rodrigues AJ, do Carmo Costa M, Silva TL, Ferreira D, Bajanca F, Logarinho E, Maciel P.

Biochim Biophys Acta. 2010 Oct;1803(10):1154-63. doi: 10.1016/j.bbamcr.2010.07.004. Epub 2010 Jul 15.


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.


Toward understanding Machado-Joseph disease.

Costa Mdo C, Paulson HL.

Prog Neurobiol. 2012 May;97(2):239-57. doi: 10.1016/j.pneurobio.2011.11.006. Epub 2011 Nov 23. Review.


A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript.

Ramani B, Harris GM, Huang R, Seki T, Murphy GG, Costa Mdo C, Fischer S, Saunders TL, Xia G, McEachin RC, Paulson HL.

Hum Mol Genet. 2015 Mar 1;24(5):1211-24. doi: 10.1093/hmg/ddu532. Epub 2014 Oct 15.


Mouse ataxin-3 functional knock-out model.

Switonski PM, Fiszer A, Kazmierska K, Kurpisz M, Krzyzosiak WJ, Figiel M.

Neuromolecular Med. 2011 Mar;13(1):54-65. doi: 10.1007/s12017-010-8137-3. Epub 2010 Oct 14.


FOXO4 induces human plasminogen activator inhibitor-1 gene expression via an indirect mechanism by modulating HIF-1alpha and CREB levels.

Dimova EY, Samoylenko A, Kietzmann T.

Antioxid Redox Signal. 2010 Aug 15;13(4):413-24. doi: 10.1089/ars.2009.2999.


Proteotoxic stress increases nuclear localization of ataxin-3.

Reina CP, Zhong X, Pittman RN.

Hum Mol Genet. 2010 Jan 15;19(2):235-49. doi: 10.1093/hmg/ddp482. Epub 2009 Oct 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.


Aqueous extract of Gardenia jasminoides targeting oxidative stress to reduce polyQ aggregation in cell models of spinocerebellar ataxia 3.

Chang KH, Chen WL, Wu YR, Lin TH, Wu YC, Chao CY, Lin JY, Lee LC, Chen YC, Lee-Chen GJ, Chen CM.

Neuropharmacology. 2014 Jun;81:166-75. doi: 10.1016/j.neuropharm.2014.01.032. Epub 2014 Jan 31.


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.


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