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Items: 1 to 20 of 54

1.

Short-term succinic acid treatment mitigates cerebellar mitochondrial OXPHOS dysfunction, neurodegeneration and ataxia in a Purkinje-specific spinocerebellar ataxia type 1 (SCA1) mouse model.

Ferro A, Carbone E, Zhang J, Marzouk E, Villegas M, Siegel A, Nguyen D, Possidente T, Hartman J, Polley K, Ingram MA, Berry G, Reynolds TH, Possidente B, Frederick K, Ives S, Lagalwar S.

PLoS One. 2017 Dec 6;12(12):e0188425. doi: 10.1371/journal.pone.0188425. eCollection 2017.

2.

Motor and Cerebellar Architectural Abnormalities during the Early Progression of Ataxia in a Mouse Model of SCA1 and How Early Prevention Leads to a Better Outcome Later in Life.

Ibrahim MF, Power EM, Potapov K, Empson RM.

Front Cell Neurosci. 2017 Sep 20;11:292. doi: 10.3389/fncel.2017.00292. eCollection 2017.

3.

Polyglutamine expansion affects huntingtin conformation in multiple Huntington's disease models.

Daldin M, Fodale V, Cariulo C, Azzollini L, Verani M, Martufi P, Spiezia MC, Deguire SM, Cherubini M, Macdonald D, Weiss A, Bresciani A, Vonsattel JG, Petricca L, Marsh JL, Gines S, Santimone I, Marano M, Lashuel HA, Squitieri F, Caricasole A.

Sci Rep. 2017 Jul 11;7(1):5070. doi: 10.1038/s41598-017-05336-7.

4.

Inhibition of colony-stimulating factor 1 receptor early in disease ameliorates motor deficits in SCA1 mice.

Qu W, Johnson A, Kim JH, Lukowicz A, Svedberg D, Cvetanovic M.

J Neuroinflammation. 2017 May 25;14(1):107. doi: 10.1186/s12974-017-0880-z.

5.

The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex.

Bott LC, Salomons FA, Maric D, Liu Y, Merry D, Fischbeck KH, Dantuma NP.

Sci Rep. 2016 Jun 17;6:27703. doi: 10.1038/srep27703.

6.

Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways.

Ingram M, Wozniak EAL, Duvick L, Yang R, Bergmann P, Carson R, O'Callaghan B, Zoghbi HY, Henzler C, Orr HT.

Neuron. 2016 Mar 16;89(6):1194-1207. doi: 10.1016/j.neuron.2016.02.011. Epub 2016 Mar 3.

7.

Preventing the Androgen Receptor N/C Interaction Delays Disease Onset in a Mouse Model of SBMA.

Zboray L, Pluciennik A, Curtis D, Liu Y, Berman-Booty LD, Orr C, Kesler CT, Berger T, Gioeli D, Paschal BM, Merry DE.

Cell Rep. 2015 Dec 15;13(10):2312-23. doi: 10.1016/j.celrep.2015.11.019. Epub 2015 Dec 7.

8.

Identification of novel polyglutamine-expanded aggregation species in spinal and bulbar muscular atrophy.

Berger TR, Montie HL, Jain P, Legleiter J, Merry DE.

Brain Res. 2015 Dec 2;1628(Pt B):254-264. doi: 10.1016/j.brainres.2015.09.033. Epub 2015 Oct 8.

9.

Consensus Paper: Cerebellar Development.

Leto K, Arancillo M, Becker EB, Buffo A, Chiang C, Ding B, Dobyns WB, Dusart I, Haldipur P, Hatten ME, Hoshino M, Joyner AL, Kano M, Kilpatrick DL, Koibuchi N, Marino S, Martinez S, Millen KJ, Millner TO, Miyata T, Parmigiani E, Schilling K, Sekerková G, Sillitoe RV, Sotelo C, Uesaka N, Wefers A, Wingate RJ, Hawkes R.

Cerebellum. 2016 Dec;15(6):789-828. Review.

10.

Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability.

Dell'Orco JM, Wasserman AH, Chopra R, Ingram MA, Hu YS, Singh V, Wulff H, Opal P, Orr HT, Shakkottai VG.

J Neurosci. 2015 Aug 12;35(32):11292-307. doi: 10.1523/JNEUROSCI.1357-15.2015.

11.

Studying polyglutamine diseases in Drosophila.

Xu Z, Tito AJ, Rui YN, Zhang S.

Exp Neurol. 2015 Dec;274(Pt A):25-41. doi: 10.1016/j.expneurol.2015.08.002. Epub 2015 Aug 6. Review.

12.

Induced pluripotent stem cell technology for modelling and therapy of cerebellar ataxia.

Watson LM, Wong MM, Becker EB.

Open Biol. 2015 Jul;5(7):150056. doi: 10.1098/rsob.150056. Review.

13.

The mutant Moonwalker TRPC3 channel links calcium signaling to lipid metabolism in the developing cerebellum.

Dulneva A, Lee S, Oliver PL, Di Gleria K, Kessler BM, Davies KE, Becker EB.

Hum Mol Genet. 2015 Jul 15;24(14):4114-25. doi: 10.1093/hmg/ddv150. Epub 2015 Apr 23.

14.

Disrupting SUMOylation enhances transcriptional function and ameliorates polyglutamine androgen receptor-mediated disease.

Chua JP, Reddy SL, Yu Z, Giorgetti E, Montie HL, Mukherjee S, Higgins J, McEachin RC, Robins DM, Merry DE, Iñiguez-Lluhí JA, Lieberman AP.

J Clin Invest. 2015 Feb;125(2):831-45. doi: 10.1172/JCI73214. Epub 2015 Jan 20.

15.

Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1.

Cvetanovic M, Ingram M, Orr H, Opal P.

Neuroscience. 2015 Mar 19;289:289-99. doi: 10.1016/j.neuroscience.2015.01.003. Epub 2015 Jan 14.

16.

Gait analysis and the cumulative gait index (CGI): Translational tools to assess impairments exhibited by rats with olivocerebellar ataxia.

Lambert CS, Philpot RM, Engberg ME, Johns BE, Kim SH, Wecker L.

Behav Brain Res. 2014 Nov 1;274:334-43. doi: 10.1016/j.bbr.2014.08.004. Epub 2014 Aug 10.

17.

The role for alterations in neuronal activity in the pathogenesis of polyglutamine repeat disorders.

Chopra R, Shakkottai VG.

Neurotherapeutics. 2014 Oct;11(4):751-63. doi: 10.1007/s13311-014-0289-7. Review.

18.

Beyond the glutamine expansion: influence of posttranslational modifications of ataxin-1 in the pathogenesis of spinocerebellar ataxia type 1.

Ju H, Kokubu H, Lim J.

Mol Neurobiol. 2014 Dec;50(3):866-874. doi: 10.1007/s12035-014-8703-z. Epub 2014 Apr 22. Review.

19.

Synapse elimination in the developing cerebellum.

Hashimoto K, Kano M.

Cell Mol Life Sci. 2013 Dec;70(24):4667-80. doi: 10.1007/s00018-013-1405-2. Epub 2013 Jun 28. Review.

20.

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.

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