{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T09:50:10Z","timestamp":1792403410774,"version":"4.4.0"},"reference-count":50,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,8,13]],"date-time":"2020-08-13T00:00:00Z","timestamp":1597276800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2020,8,13]],"date-time":"2020-08-13T00:00:00Z","timestamp":1597276800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["U01-NS106670"],"award-info":[{"award-number":["U01-NS106670"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R01-NS085054"],"award-info":[{"award-number":["R01-NS085054"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Cerebellum"],"published-print":{"date-parts":[[2021,2]]},"DOI":"10.1007\/s12311-020-01179-7","type":"journal-article","created":{"date-parts":[[2020,8,13]],"date-time":"2020-08-13T02:03:01Z","timestamp":1597284181000},"page":"41-53","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Antisense Oligonucleotide Therapy Targeted Against ATXN3 Improves Potassium Channel\u2013Mediated Purkinje Neuron Dysfunction in Spinocerebellar Ataxia Type 3"],"prefix":"10.1007","volume":"20","author":[{"given":"David D.","family":"Bushart","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annie J.","family":"Zalon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongjiu","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Logan M.","family":"Morrison","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuanfang","family":"Guan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henry L.","family":"Paulson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vikram G.","family":"Shakkottai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4279-2758","authenticated-orcid":false,"given":"Hayley S.","family":"McLoughlin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,8,13]]},"reference":[{"issue":"9","key":"1179_CR1","doi-asserted-by":"publisher","first-page":"885","DOI":"10.1016\/S1474-4422(10)70183-6","volume":"9","author":"A Durr","year":"2010","unstructured":"Durr A. Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond. Lancet Neurol. 2010;9(9):885\u201394.","journal-title":"Lancet Neurol"},{"issue":"6","key":"1179_CR2","doi-asserted-by":"publisher","first-page":"650","DOI":"10.1001\/jamaneurol.2019.0423","volume":"76","author":"SL Gardiner","year":"2019","unstructured":"Gardiner SL, Boogaard MW, Trompet S, de Mutsert R, Rosendaal FR, Gussekloo J, et al. Prevalence of carriers of intermediate and pathological polyglutamine disease-associated alleles among large population-based cohorts. JAMA Neurol. 2019;76(6):650\u20136.","journal-title":"JAMA Neurol"},{"key":"1179_CR3","doi-asserted-by":"publisher","first-page":"104635","DOI":"10.1016\/j.nbd.2019.104635","volume":"134","author":"HS McLoughlin","year":"2020","unstructured":"McLoughlin HS, Moore LR, Paulson HL. Pathogenesis of SCA3 and implications for other polyglutamine diseases. Neurobiol Dis. 2020;134:104635.","journal-title":"Neurobiol Dis"},{"issue":"1","key":"1179_CR4","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s00401-012-1000-x","volume":"124","author":"K Seidel","year":"2012","unstructured":"Seidel K, Siswanto S, Brunt ERP, den Dunnen W, Korf HW, Rub U. Brain pathology of spinocerebellar ataxias. Acta Neuropathol. 2012;124(1):1\u201321.","journal-title":"Acta Neuropathol"},{"key":"1179_CR5","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1016\/j.pneurobio.2013.01.001","volume":"104","author":"U Rub","year":"2013","unstructured":"Rub U, Schols L, Paulson H, Auburger G, Kermer P, Jen JC, et al. Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7. Prog Neurobiol. 2013;104:38\u201366.","journal-title":"Prog Neurobiol"},{"issue":"10","key":"1179_CR6","doi-asserted-by":"publisher","first-page":"613","DOI":"10.1038\/nrn.2017.92","volume":"18","author":"HL Paulson","year":"2017","unstructured":"Paulson HL, Shakkottai VG, Clark HB, Orr HT. Polyglutamine spinocerebellar ataxias - from genes to potential treatments. Nat Rev Neurosci. 2017;18(10):613\u201326.","journal-title":"Nat Rev Neurosci"},{"issue":"1","key":"1179_CR7","doi-asserted-by":"publisher","first-page":"54","DOI":"10.1007\/s12017-010-8137-3","volume":"13","author":"PM Switonski","year":"2011","unstructured":"Switonski PM, Fiszer A, Kazmierska K, Kurpisz M, Krzyzosiak WJ, Figiel M. Mouse ataxin-3 functional knock-out model. NeuroMolecular Med. 2011;13(1):54\u201365.","journal-title":"NeuroMolecular Med"},{"issue":"1","key":"1179_CR8","doi-asserted-by":"publisher","first-page":"64","DOI":"10.1002\/ana.25264","volume":"84","author":"HS McLoughlin","year":"2018","unstructured":"McLoughlin HS, Moore LR, Chopra R, Komlo R, McKenzie M, Blumenstein KG, et al. Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice. Ann Neurol. 2018;84(1):64\u201377.","journal-title":"Ann Neurol"},{"key":"1179_CR9","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1016\/j.omtn.2017.04.005","volume":"7","author":"LR Moore","year":"2017","unstructured":"Moore LR, Rajpal G, Dillingham IT, Qutob M, Blumenstein KG, Gattis D, et al. Evaluation of antisense oligonucleotides targeting ATXN3 in SCA3 mouse models. Mol Ther-Nucl Acids. 2017;7:200\u201310.","journal-title":"Mol Ther-Nucl Acids"},{"key":"1179_CR10","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1016\/j.omtn.2017.06.019","volume":"8","author":"LJA Toonen","year":"2017","unstructured":"Toonen LJA, Rigo F, van Attikum H, van Roon-Mom WMC. Antisense oligonucleotide-mediated removal of the polyglutamine repeat in spinocerebellar ataxia type 3 mice. Mol Ther-Nucl Acids. 2017;8:232\u201342.","journal-title":"Mol Ther-Nucl Acids"},{"issue":"32","key":"1179_CR11","doi-asserted-by":"publisher","first-page":"11292","DOI":"10.1523\/JNEUROSCI.1357-15.2015","volume":"35","author":"JM Dell\u2019Orco","year":"2015","unstructured":"Dell\u2019Orco JM, Wasserman AH, Chopra R, Ingram MAC, Hu YS, Singh V, et al. Neuronal atrophy early in degenerative ataxia is a compensatory mechanism to regulate membrane excitability. J Neurosci. 2015;35(32):11292\u2013307.","journal-title":"J Neurosci"},{"issue":"20","key":"1179_CR12","doi-asserted-by":"publisher","first-page":"3935","DOI":"10.1093\/hmg\/ddx281","volume":"26","author":"JM Dell\u2019Orco","year":"2017","unstructured":"Dell\u2019Orco JM, Pulst SM, Shakkottai VG. Potassium channel dysfunction underlies Purkinje neuron spiking abnormalities in spinocerebellar ataxia type 2. Hum Mol Genet. 2017;26(20):3935\u201345.","journal-title":"Hum Mol Genet"},{"issue":"2","key":"1179_CR13","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1093\/hmg\/dds427","volume":"22","author":"ST Hansen","year":"2013","unstructured":"Hansen ST, Meera P, Otis TS, Pulst SM. Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2. Hum Mol Genet. 2013;22(2):271\u201383.","journal-title":"Hum Mol Genet"},{"issue":"36","key":"1179_CR14","doi-asserted-by":"publisher","first-page":"13002","DOI":"10.1523\/JNEUROSCI.2789-11.2011","volume":"31","author":"VG Shakkottai","year":"2011","unstructured":"Shakkottai VG, do Carmo Costa M, Dell\u2019Orco JM, Sankaranarayanan A, Wulff H, Paulson HL. Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3. J Neurosci. 2011;31(36):13002\u201314.","journal-title":"J Neurosci"},{"key":"1179_CR15","doi-asserted-by":"publisher","first-page":"29489","DOI":"10.1038\/srep29489","volume":"6","author":"S Jayabal","year":"2016","unstructured":"Jayabal S, Chang HH, Cullen KE, Watt AJ. 4-aminopyridine reverses ataxia and cerebellar firing deficiency in a mouse model of spinocerebellar ataxia type 6. Sci Rep. 2016;6:29489.","journal-title":"Sci Rep"},{"issue":"4","key":"1179_CR16","doi-asserted-by":"publisher","first-page":"630","DOI":"10.1016\/j.neuron.2019.11.019","volume":"105","author":"CA Stoyas","year":"2020","unstructured":"Stoyas CA, Bushart DD, Switonski PM, Ward JM, Alaghatta A, Tang MB, et al. Nicotinamide pathway-dependent Sirt1 activation restores calcium homeostasis to achieve neuroprotection in spinocerebellar ataxia type 7. Neuron. 2020;105(4):630\u2013+.","journal-title":"Neuron"},{"key":"1179_CR17","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/j.neulet.2018.02.005","volume":"688","author":"DD Bushart","year":"2019","unstructured":"Bushart DD, Shakkottai VG. Ion channel dysfunction in cerebellar ataxia. Neurosci Lett. 2019;688:41\u20138.","journal-title":"Neurosci Lett"},{"issue":"9","key":"1179_CR18","doi-asserted-by":"publisher","first-page":"1075","DOI":"10.1093\/hmg\/11.9.1075","volume":"11","author":"CK Cemal","year":"2002","unstructured":"Cemal CK, Carroll CJ, Lawrence L, Lowrie MB, Ruddle P, Al-Mahdawi S, et al. YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit. Hum Mol Genet. 2002;11(9):1075\u201394.","journal-title":"Hum Mol Genet"},{"key":"1179_CR19","unstructured":"\u00a9 2004 Allen Institute for Brain Science, Allen Mouse Brain Atlas. Available from: mouse.brain-map.org, Lein ES, Hawrylycz MJ, Ao N, Ayres M, et al. Genome-wide atlas of gene expression in the adult mouse brain. Nature. 2007;445(7124):168\u201376."},{"issue":"3","key":"1179_CR20","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1002\/acn3.527","volume":"5","author":"DD Bushart","year":"2018","unstructured":"Bushart DD, Chopra R, Singh V, Murphy GG, Wulff H, Shakkottai VG. Targeting potassium channels to treat cerebellar ataxia. Ann Clin Transl Neurol. 2018;5(3):297\u2013314.","journal-title":"Ann Clin Transl Neurol"},{"issue":"1\u20132","key":"1179_CR21","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1016\/S0006-8993(01)02068-6","volume":"895","author":"YH Chung","year":"2001","unstructured":"Chung YH, Shin C, Kim MJ, Lee BK, Cha CI. Immunohistochemical study on the distribution of six members of the Kv1 channel subunits in the rat cerebellum. Brain Res. 2001;895(1\u20132):173\u20137.","journal-title":"Brain Res"},{"issue":"2","key":"1179_CR22","doi-asserted-by":"publisher","first-page":"1081","DOI":"10.1152\/jn.1998.79.2.1081","volume":"79","author":"P Serodio","year":"1998","unstructured":"Serodio P, Rudy B. Differential expression of Kv4 K+ channel subunits mediating subthreshold transient K+ (A-type) currents in rat brain. J Neurophysiol. 1998;79(2):1081\u201391.","journal-title":"J Neurophysiol"},{"issue":"1","key":"1179_CR23","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1523\/JNEUROSCI.20-01-00114.2000","volume":"20","author":"AP Southan","year":"2000","unstructured":"Southan AP, Robertson B. Electrophysiological characterization of voltage-gated K(+) currents in cerebellar basket and purkinje cells: Kv1 and Kv3 channel subfamilies are present in basket cell nerve terminals. J Neurosci. 2000;20(1):114\u201322.","journal-title":"J Neurosci"},{"issue":"50","key":"1179_CR24","doi-asserted-by":"publisher","first-page":"15735","DOI":"10.1523\/JNEUROSCI.4048-09.2009","volume":"29","author":"EC Hurlock","year":"2009","unstructured":"Hurlock EC, Bose M, Pierce G, Joho RH. Rescue of motor coordination by Purkinje cell-targeted restoration of Kv3.3 channels in Kcnc3-null mice requires Kcnc1. J Neurosci. 2009;29(50):15735\u201344.","journal-title":"J Neurosci"},{"issue":"2","key":"1179_CR25","doi-asserted-by":"publisher","first-page":"136","DOI":"10.1038\/ng1094-136","volume":"8","author":"DL Browne","year":"1994","unstructured":"Browne DL, Gancher ST, Nutt JG, Brunt ER, Smith EA, Kramer P, et al. Episodic ataxia\/myokymia syndrome is associated with point mutations in the human potassium channel gene, KCNA1. Nat Genet. 1994;8(2):136\u201340.","journal-title":"Nat Genet"},{"issue":"4","key":"1179_CR26","doi-asserted-by":"publisher","first-page":"447","DOI":"10.1038\/ng1758","volume":"38","author":"MF Waters","year":"2006","unstructured":"Waters MF, Minassian NA, Stevanin G, Figueroa KP, Bannister JP, Nolte D, et al. Mutations in voltage-gated potassium channel KCNC3 cause degenerative and developmental central nervous system phenotypes. Nat Genet. 2006;38(4):447\u201351.","journal-title":"Nat Genet"},{"issue":"6","key":"1179_CR27","doi-asserted-by":"publisher","first-page":"859","DOI":"10.1002\/ana.23701","volume":"72","author":"YC Lee","year":"2012","unstructured":"Lee YC, Durr A, Majczenko K, Huang YH, Liu YC, Lien CC, et al. Mutations in KCND3 cause spinocerebellar ataxia type 22. Ann Neurol. 2012;72(6):859\u201369.","journal-title":"Ann Neurol"},{"issue":"6","key":"1179_CR28","doi-asserted-by":"publisher","first-page":"870","DOI":"10.1002\/ana.23700","volume":"72","author":"A Duarri","year":"2012","unstructured":"Duarri A, Jezierska J, Fokkens M, Meijer M, Schelhaas HJ, den Dunnen WF, et al. Mutations in potassium channel kcnd3 cause spinocerebellar ataxia type 19. Ann Neurol. 2012;72(6):870\u201380.","journal-title":"Ann Neurol"},{"issue":"10","key":"1179_CR29","doi-asserted-by":"publisher","first-page":"1898","DOI":"10.1038\/mt.2013.144","volume":"21","author":"C Costa Mdo","year":"2013","unstructured":"Costa Mdo C, Luna-Cancalon K, Fischer S, Ashraf NS, Ouyang M, Dharia RM, et al. Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease. Mol Ther. 2013;21(10):1898\u2013908.","journal-title":"Mol Ther"},{"issue":"1","key":"1179_CR30","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1038\/s41572-019-0074-3","volume":"5","author":"T Klockgether","year":"2019","unstructured":"Klockgether T, Mariotti C, Paulson HL. Spinocerebellar ataxia. Nat Rev Dis Primers. 2019;5(1):24.","journal-title":"Nat Rev Dis Primers"},{"key":"1179_CR31","first-page":"580","volume-title":"The cerebellum and neural control","author":"It*o M","year":"1984","unstructured":"It*o M. The cerebellum and neural control, vol. xvii. New York: Raven Press; 1984. p. 580."},{"key":"1179_CR32","doi-asserted-by":"publisher","first-page":"e02536","DOI":"10.7554\/eLife.02536","volume":"3","author":"H Zhou","year":"2014","unstructured":"Zhou H, Lin Z, Voges K, Ju C, Gao Z, Bosman LW, et al. Cerebellar modules operate at different frequencies. Elife. 2014;3:e02536.","journal-title":"Elife."},{"issue":"12","key":"1179_CR33","doi-asserted-by":"publisher","first-page":"3317","DOI":"10.1111\/j.0953-816X.2004.03385.x","volume":"19","author":"A McMahon","year":"2004","unstructured":"McMahon A, Fowler SC, Perney TM, Akemann W, Knopfel T, Joho RH. Allele-dependent changes of olivocerebellar circuit properties in the absence of the voltage-gated potassium channels Kv3.1 and Kv3.3. Eur J Neurosci. 2004;19(12):3317\u201327.","journal-title":"Eur J Neurosci"},{"issue":"17","key":"1179_CR34","doi-asserted-by":"publisher","first-page":"4602","DOI":"10.1523\/JNEUROSCI.5204-05.2006","volume":"26","author":"W Akemann","year":"2006","unstructured":"Akemann W, Knopfel T. Interaction of Kv3 potassium channels and resurgent sodium current influences the rate of spontaneous firing of Purkinje neurons. J Neurosci. 2006;26(17):4602\u201312.","journal-title":"J Neurosci"},{"issue":"6","key":"1179_CR35","doi-asserted-by":"publisher","first-page":"3516","DOI":"10.1152\/jn.00982.2009","volume":"103","author":"E Zagha","year":"2010","unstructured":"Zagha E, Manita S, Ross WN, Rudy B. Dendritic Kv3.3 potassium channels in cerebellar purkinje cells regulate generation and spatial dynamics of dendritic Ca2+ spikes. J Neurophysiol. 2010;103(6):3516\u201325.","journal-title":"J Neurophysiol"},{"issue":"Pt 5","key":"1179_CR36","doi-asserted-by":"publisher","first-page":"1182","DOI":"10.1093\/brain\/awv064","volume":"138","author":"MR Stefanescu","year":"2015","unstructured":"Stefanescu MR, Dohnalek M, Maderwald S, Thurling M, Minnerop M, Beck A, et al. Structural and functional MRI abnormalities of cerebellar cortex and nuclei in SCA3, SCA6 and Friedreich\u2019s ataxia. Brain. 2015;138(Pt 5):1182\u201397.","journal-title":"Brain."},{"issue":"3","key":"1179_CR37","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1002\/ana.25297","volume":"84","author":"TJR Rezende","year":"2018","unstructured":"Rezende TJR, de Paiva JLR, Martinez ARM, Lopes-Cendes I, Pedroso JL, Barsottini OGP, et al. Structural signature of SCA3: from presymptomatic to late disease stages. Ann Neurol. 2018;84(3):401\u20138.","journal-title":"Ann Neurol"},{"issue":"10","key":"1179_CR38","doi-asserted-by":"publisher","first-page":"590","DOI":"10.1038\/s41582-018-0051-6","volume":"14","author":"T Ashizawa","year":"2018","unstructured":"Ashizawa T, Oz G, Paulson HL. Spinocerebellar ataxias: prospects and challenges for therapy development. Nat Rev Neurol. 2018;14(10):590\u2013605.","journal-title":"Nat Rev Neurol"},{"issue":"5","key":"1179_CR39","doi-asserted-by":"publisher","first-page":"660","DOI":"10.1111\/jnc.12435","volume":"127","author":"UE Emir","year":"2013","unstructured":"Emir UE, Brent Clark H, Vollmers ML, Eberly LE, Oz G. Non-invasive detection of neurochemical changes prior to overt pathology in a mouse model of spinocerebellar ataxia type 1. J Neurochem. 2013;127(5):660\u20138.","journal-title":"J Neurochem"},{"issue":"10","key":"1179_CR40","doi-asserted-by":"publisher","first-page":"3831","DOI":"10.1523\/JNEUROSCI.5612-09.2010","volume":"30","author":"G Oz","year":"2010","unstructured":"Oz G, Nelson CD, Koski DM, Henry PG, Marjanska M, Deelchand DK, et al. Noninvasive detection of presymptomatic and progressive neurodegeneration in a mouse model of spinocerebellar ataxia type 1. J Neurosci. 2010;30(10):3831\u20138.","journal-title":"J Neurosci"},{"key":"1179_CR41","doi-asserted-by":"crossref","unstructured":"Friedrich J, Kordasiewicz HB, O\u2019Callaghan B, Handler HP, Wagener C, Duvick L, et al. Antisense oligonucleotide-mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles. JCI Insight. 2018;3(21).","DOI":"10.1172\/jci.insight.123193"},{"issue":"7","key":"1179_CR42","doi-asserted-by":"publisher","first-page":"549","DOI":"10.1002\/mdc3.12804","volume":"6","author":"DK Deelchand","year":"2019","unstructured":"Deelchand DK, Joers JM, Ravishankar A, Lyu T, Emir UE, Hutter D, et al. Sensitivity of volumetric magnetic resonance imaging and magnetic resonance spectroscopy to progression of spinocerebellar ataxia type 1. Mov Disord Clin Pract. 2019;6(7):549\u201358.","journal-title":"Mov Disord Clin Pract"},{"issue":"5","key":"1179_CR43","doi-asserted-by":"publisher","first-page":"662","DOI":"10.1002\/mds.26181","volume":"30","author":"IM Adanyeguh","year":"2015","unstructured":"Adanyeguh IM, Henry PG, Nguyen TM, Rinaldi D, Jauffret C, Valabregue R, et al. In vivo neurometabolic profiling in patients with spinocerebellar ataxia types 1, 2, 3, and 7. Mov Disord. 2015;30(5):662\u201370.","journal-title":"Mov Disord"},{"issue":"4","key":"1179_CR44","doi-asserted-by":"publisher","first-page":"816","DOI":"10.1002\/ana.25212","volume":"83","author":"JM Joers","year":"2018","unstructured":"Joers JM, Deelchand DK, Lyu T, Emir UE, Hutter D, Gomez CM, et al. Neurochemical abnormalities in premanifest and early spinocerebellar ataxias. Ann Neurol. 2018;83(4):816\u201329.","journal-title":"Ann Neurol"},{"key":"1179_CR45","doi-asserted-by":"publisher","DOI":"10.1002\/mds.28140","volume-title":"Mov Disord","author":"MC Costa","year":"2020","unstructured":"Costa MC, Radzwion M, McLoughlin HS, Ashraf NS, Fischer S, Shakkottai VG, et al. In vivo molecular signatures of cerebellar pathology in spinocerebellar ataxia type 3. In: Mov Disord; 2020. https:\/\/doi.org\/10.1002\/mds.28140."},{"issue":"6","key":"1179_CR46","doi-asserted-by":"publisher","first-page":"1449","DOI":"10.1016\/0896-6273(95)90022-5","volume":"15","author":"JP Adelman","year":"1995","unstructured":"Adelman JP, Bond CT, Pessia M, Maylie J. Episodic ataxia results from voltage-dependent potassium channels with altered functions. Neuron. 1995;15(6):1449\u201354.","journal-title":"Neuron."},{"issue":"1","key":"1179_CR47","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/S0169-328X(96)00120-9","volume":"42","author":"G Laube","year":"1996","unstructured":"Laube G, Roper J, Pitt JC, Sewing S, Kistner U, Garner CC, et al. Ultrastructural localization of shaker-related potassium channel subunits and synapse-associated protein 90 to septate-like junctions in rat cerebellar Pinceaux. Brain Res Mol Brain Res. 1996;42(1):51\u201361.","journal-title":"Brain Res Mol Brain Res"},{"issue":"32","key":"1179_CR48","doi-asserted-by":"publisher","first-page":"11433","DOI":"10.1523\/JNEUROSCI.1346-15.2015","volume":"35","author":"MJ Kole","year":"2015","unstructured":"Kole MJ, Qian J, Waase MP, Klassen TL, Chen TT, Augustine GJ, et al. Selective loss of presynaptic potassium channel clusters at the cerebellar basket cell terminal pinceau in Adam11 mutants reveals their role in ephaptic control of Purkinje cell firing. J Neurosci. 2015;35(32):11433\u201344.","journal-title":"J Neurosci"},{"issue":"Pt 9","key":"1179_CR49","doi-asserted-by":"publisher","first-page":"2444","DOI":"10.1093\/brain\/awu174","volume":"137","author":"S Tezenas du Montcel","year":"2014","unstructured":"Tezenas du Montcel S, Durr A, Bauer P, Figueroa KP, Ichikawa Y, Brussino A, et al. Modulation of the age at onset in spinocerebellar ataxia by CAG tracts in various genes. Brain. 2014;137(Pt 9):2444\u201355.","journal-title":"Brain."},{"issue":"16","key":"1179_CR50","doi-asserted-by":"publisher","first-page":"3232","DOI":"10.1093\/hmg\/ddx176","volume":"26","author":"B Ramani","year":"2017","unstructured":"Ramani B, Harris GM, Huang R, Seki T, Murphy GG, Carmo Costa MD, et al. A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript. Hum Mol Genet. 2017;26(16):3232\u20133.","journal-title":"Hum Mol Genet"}],"container-title":["The Cerebellum"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12311-020-01179-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12311-020-01179-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12311-020-01179-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,11,6]],"date-time":"2022-11-06T14:58:59Z","timestamp":1667746739000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12311-020-01179-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,13]]},"references-count":50,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,2]]}},"alternative-id":["1179"],"URL":"https:\/\/doi.org\/10.1007\/s12311-020-01179-7","relation":{},"ISSN":["1473-4222","1473-4230"],"issn-type":[{"value":"1473-4222","type":"print"},{"value":"1473-4230","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,8,13]]},"assertion":[{"value":"13 August 2020","order":1,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"All animal procedures were approved by the University of Michigan Institutional Animal Care and Use Committee (IACUC). Procedures were conducted in accordance with the U.S. Public Health Service\u2019s Policy on Humane Care and Use of Laboratory Animals.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with Ethical Standards"}},{"value":"The authors declare that they have no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}}]}}