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Items: 1 to 20 of 114


Antisense oligonucleotide therapy for spinocerebellar ataxia type 2.

Scoles DR, Meera P, Schneider MD, Paul S, Dansithong W, Figueroa KP, Hung G, Rigo F, Bennett CF, Otis TS, Pulst SM.

Nature. 2017 Apr 20;544(7650):362-366. doi: 10.1038/nature22044. Epub 2017 Apr 12.


Ataxin-2 regulates RGS8 translation in a new BAC-SCA2 transgenic mouse model.

Dansithong W, Paul S, Figueroa KP, Rinehart MD, Wiest S, Pflieger LT, Scoles DR, Pulst SM.

PLoS Genet. 2015 Apr 22;11(4):e1005182. doi: 10.1371/journal.pgen.1005182. eCollection 2015 Apr.


Spinocerebellar Ataxia Type 2.

Scoles DR, Pulst SM.

Adv Exp Med Biol. 2018;1049:175-195. doi: 10.1007/978-3-319-71779-1_8. Review.


Oligonucleotide therapeutics in neurodegenerative diseases.

Scoles DR, Pulst SM.

RNA Biol. 2018;15(6):707-714. doi: 10.1080/15476286.2018.1454812. Epub 2018 Jun 1. Review.


ATXN2-AS, a gene antisense to ATXN2, is associated with spinocerebellar ataxia type 2 and amyotrophic lateral sclerosis.

Li PP, Sun X, Xia G, Arbez N, Paul S, Zhu S, Peng HB, Ross CA, Koeppen AH, Margolis RL, Pulst SM, Ashizawa T, Rudnicki DD.

Ann Neurol. 2016 Oct;80(4):600-15. doi: 10.1002/ana.24761.


Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2.

Hansen ST, Meera P, Otis TS, Pulst SM.

Hum Mol Genet. 2013 Jan 15;22(2):271-83. doi: 10.1093/hmg/dds427. Epub 2012 Oct 18.


Degenerative ataxias, from genes to therapies: The 2015 Cotzias Lecture.

Pulst SM.

Neurology. 2016 Jun 14;86(24):2284-90. doi: 10.1212/WNL.0000000000002777. Review.


Gene co-expression network analysis for identifying modules and functionally enriched pathways in SCA2.

Pflieger LT, Dansithong W, Paul S, Scoles DR, Figueroa KP, Meera P, Otis TS, Facelli JC, Pulst SM.

Hum Mol Genet. 2017 Aug 15;26(16):3069-3080. doi: 10.1093/hmg/ddx191.


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.


Mesenchymal stem cell transplantation ameliorates motor function deterioration of spinocerebellar ataxia by rescuing cerebellar Purkinje cells.

Chang YK, Chen MH, Chiang YH, Chen YF, Ma WH, Tseng CY, Soong BW, Ho JH, Lee OK.

J Biomed Sci. 2011 Aug 8;18:54. doi: 10.1186/1423-0127-18-54.


Protein kinase C activity is a protective modifier of Purkinje neuron degeneration in cerebellar ataxia.

Chopra R, Wasserman AH, Pulst SM, De Zeeuw CI, Shakkottai VG.

Hum Mol Genet. 2018 Apr 15;27(8):1396-1410. doi: 10.1093/hmg/ddy050.


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.


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.


Atxn2 Knockout and CAG42-Knock-in Cerebellum Shows Similarly Dysregulated Expression in Calcium Homeostasis Pathway.

Halbach MV, Gispert S, Stehning T, Damrath E, Walter M, Auburger G.

Cerebellum. 2017 Feb;16(1):68-81. doi: 10.1007/s12311-016-0762-4.


[Advance in research on spinocerebellar ataxia 2].

Jing F, Yang D, Chen T.

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2018 Apr 10;35(2):284-287. doi: 10.3760/cma.j.issn.1003-9406.2018.02.032. Review. Chinese.


Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration.

Paul S, Dansithong W, Figueroa KP, Scoles DR, Pulst SM.

Nat Commun. 2018 Sep 7;9(1):3648. doi: 10.1038/s41467-018-06041-3.


Pharmacological enhancement of mGlu1 metabotropic glutamate receptors causes a prolonged symptomatic benefit in a mouse model of spinocerebellar ataxia type 1.

Notartomaso S, Zappulla C, Biagioni F, Cannella M, Bucci D, Mascio G, Scarselli P, Fazio F, Weisz F, Lionetto L, Simmaco M, Gradini R, Battaglia G, Signore M, Puliti A, Nicoletti F.

Mol Brain. 2013 Nov 19;6:48. doi: 10.1186/1756-6606-6-48.


Search for SCA2 blood RNA biomarkers highlights Ataxin-2 as strong modifier of the mitochondrial factor PINK1 levels.

Sen NE, Drost J, Gispert S, Torres-Odio S, Damrath E, Klinkenberg M, Hamzeiy H, Akdal G, Güllüoğlu H, Başak AN, Auburger G.

Neurobiol Dis. 2016 Dec;96:115-126. doi: 10.1016/j.nbd.2016.09.002. Epub 2016 Sep 3.


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.

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