{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T12:53:54Z","timestamp":1791550434721,"version":"4.3.4"},"reference-count":196,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2020,2,1]],"date-time":"2020-02-01T00:00:00Z","timestamp":1580515200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,2,1]],"date-time":"2020-02-01T00:00:00Z","timestamp":1580515200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2019,11,27]],"date-time":"2019-11-27T00:00:00Z","timestamp":1574812800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100002243","name":"National Ataxia Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002243","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01-NS038712"],"award-info":[{"award-number":["R01-NS038712"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["U01-NS106670"],"award-info":[{"award-number":["U01-NS106670"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R21-NS111154"],"award-info":[{"award-number":["R21-NS111154"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2020,2]]},"DOI":"10.1016\/j.nbd.2019.104635","type":"journal-article","created":{"date-parts":[[2019,10,24]],"date-time":"2019-10-24T12:17:14Z","timestamp":1571919434000},"page":"104635","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":179,"special_numbering":"C","title":["Pathogenesis of SCA3 and implications for other polyglutamine diseases"],"prefix":"10.1016","volume":"134","author":[{"given":"Hayley S.","family":"McLoughlin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren R.","family":"Moore","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0382-7535","authenticated-orcid":false,"given":"Henry L.","family":"Paulson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2019.104635_bb0005","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1002\/mds.26181","article-title":"In vivo neurometabolic profiling in patients with spinocerebellar ataxia types 1, 2, 3, and 7","volume":"30","author":"Adanyeguh","year":"2015","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2019.104635_bb0010","doi-asserted-by":"crossref","first-page":"1950","DOI":"10.1016\/j.bbadis.2015.06.010","article-title":"SUMOylation of the brain-predominant Ataxin-3 isoform modulates its interaction with p97","volume":"1852","author":"Almeida","year":"2015","journal-title":"Biochim Biophys Acta"},{"key":"10.1016\/j.nbd.2019.104635_bb0015","doi-asserted-by":"crossref","first-page":"e3341","DOI":"10.1371\/journal.pone.0003341","article-title":"Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease","volume":"3","author":"Alves","year":"2008","journal-title":"PloS One"},{"key":"10.1016\/j.nbd.2019.104635_bb0020","doi-asserted-by":"crossref","first-page":"2380","DOI":"10.1093\/hmg\/ddq111","article-title":"Silencing ataxin-3 mitigates degeneration in a rat model of Machado-Joseph disease: no role for wild-type ataxin-3?","volume":"19","author":"Alves","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0025","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1111\/pai.13020","article-title":"Ataxia-telangiectasia: a review of clinical features and molecular pathology","volume":"30","author":"Amirifar","year":"2019","journal-title":"Pediatr. Allergy Immunol."},{"key":"10.1016\/j.nbd.2019.104635_bb0030","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1016\/j.nbd.2009.07.020","article-title":"Identification and functional dissection of localization signals within ataxin-3","volume":"36","author":"Antony","year":"2009","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2019.104635_bb0035","doi-asserted-by":"crossref","first-page":"2928","DOI":"10.1093\/hmg\/ddr197","article-title":"FOXO4-dependent upregulation of superoxide dismutase-2 in response to oxidative stress is impaired in spinocerebellar ataxia type 3","volume":"20","author":"Araujo","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0040","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1038\/s41582-018-0051-6","article-title":"Spinocerebellar ataxias: prospects and challenges for therapy development","volume":"14","author":"Ashizawa","year":"2018","journal-title":"Nat. Rev. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0045","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1038\/nature22078","article-title":"Polyglutamine tracts regulate beclin 1-dependent autophagy","volume":"545","author":"Ashkenazi","year":"2017","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2019.104635_bb0050","doi-asserted-by":"crossref","first-page":"485","DOI":"10.3389\/fneur.2017.00485","article-title":"Peripheral Oxidative Stress Biomarkers in Spinocerebellar Ataxia Type 3\/Machado-Joseph Disease","volume":"8","author":"de Assis","year":"2017","journal-title":"Front Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0055","doi-asserted-by":"crossref","first-page":"3626","DOI":"10.1016\/j.cub.2017.10.054","article-title":"Stall in canonical autophagy-lysosome pathways prompts nucleophagy-based nuclear breakdown in neurodegeneration","volume":"27","author":"Baron","year":"2017","journal-title":"Curr. Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0060","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1146\/annurev-neuro-070918-050501","article-title":"Antisense oligonucleotide therapies for neurodegenerative diseases","volume":"42","author":"Bennett","year":"2019","journal-title":"Annu. Rev. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0065","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1002\/ana.24656","article-title":"DNA repair pathways underlie a common genetic mechanism modulating onset in polyglutamine diseases","volume":"79","author":"Bettencourt","year":"2016","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0070","doi-asserted-by":"crossref","first-page":"7418","DOI":"10.1523\/JNEUROSCI.4540-06.2007","article-title":"Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence","volume":"27","author":"Bichelmeier","year":"2007","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0075","doi-asserted-by":"crossref","first-page":"1950","DOI":"10.1371\/journal.pgen.0030177","article-title":"Genome-wide screen for modifiers of ataxin-3 neurodegeneration in Drosophila","volume":"3","author":"Bilen","year":"2007","journal-title":"PLoS Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0080","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.biocel.2014.03.015","article-title":"Interactions of ataxin-3 with its molecular partners in the protein machinery that sorts protein aggregates to the aggresome","volume":"51","author":"Bonanomi","year":"2014","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0085","doi-asserted-by":"crossref","first-page":"4282","DOI":"10.1093\/hmg\/ddp381","article-title":"Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3","volume":"18","author":"Boy","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0090","doi-asserted-by":"crossref","first-page":"22480","DOI":"10.1073\/pnas.0911503106","article-title":"Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms","volume":"106","author":"Bradford","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"10.1016\/j.nbd.2019.104635_bb0095","doi-asserted-by":"crossref","first-page":"938","DOI":"10.1016\/j.freeradbiomed.2004.01.003","article-title":"Treatment with a catalytic antioxidant corrects the neurobehavioral defect in ataxia-telangiectasia mice","volume":"36","author":"Browne","year":"2004","journal-title":"Free Radic Biol. Med."},{"key":"10.1016\/j.nbd.2019.104635_bb0100","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1007\/s13311-018-00696-y","article-title":"Genetics, mechanisms, and therapeutic progress in polyglutamine spinocerebellar ataxias","volume":"16","author":"Buijsen","year":"2019","journal-title":"Neurotherapeutics"},{"key":"10.1016\/j.nbd.2019.104635_bb0105","doi-asserted-by":"crossref","first-page":"4330","DOI":"10.1073\/pnas.0407252102","article-title":"The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation","volume":"102","author":"Burnett","year":"2005","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"10.1016\/j.nbd.2019.104635_bb0110","doi-asserted-by":"crossref","first-page":"3195","DOI":"10.1093\/hmg\/ddg344","article-title":"The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity","volume":"12","author":"Burnett","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0115","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/S0092-8674(02)01247-3","article-title":"DNA single-strand break repair and spinocerebellar ataxia","volume":"112","author":"Caldecott","year":"2003","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2019.104635_bb0120","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1016\/j.pediatrneurol.2007.12.006","article-title":"Homozygosity enhances severity in spinocerebellar ataxia type 3","volume":"38","author":"Carvalho","year":"2008","journal-title":"Pediatr. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0125","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1093\/hmg\/11.9.1075","article-title":"YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit","volume":"11","author":"Cemal","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0130","doi-asserted-by":"crossref","first-page":"10338","DOI":"10.1523\/JNEUROSCI.19-23-10338.1999","article-title":"Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease","volume":"19","author":"Chai","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0135","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1093\/hmg\/8.4.673","article-title":"Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3\/MJD and suppression of polyglutamine aggregation in vitro","volume":"8","author":"Chai","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0140","doi-asserted-by":"crossref","first-page":"2811","DOI":"10.1093\/hmg\/9.19.2811","article-title":"Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila","volume":"9","author":"Chan","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0145","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1004749","article-title":"The role of the mammalian DNA end-processing enzyme polynucleotide kinase 3\u2019-phosphatase in spinocerebellar ataxia type 3 pathogenesis","volume":"11","author":"Chatterjee","year":"2015","journal-title":"PLoS Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0150","doi-asserted-by":"crossref","first-page":"1166","DOI":"10.1038\/s41598-018-37774-2","article-title":"Modeling spinocerebellar ataxias 2 and 3 with iPSCs reveals a role for glutamate in disease pathology","volume":"9","author":"Chuang","year":"2019","journal-title":"Sci. Rep."},{"key":"10.1016\/j.nbd.2019.104635_bb0155","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/j.pneurobio.2011.11.006","article-title":"Toward understanding Machado-Joseph Disease","volume":"97","author":"Costa","year":"2012","journal-title":"Prog.Neurobiol."},{"key":"10.1016\/j.nbd.2019.104635_bb0160","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1038\/mt.2013.144","article-title":"Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease","volume":"21","author":"Costa","year":"2013","journal-title":"Mol. Ther."},{"key":"10.1016\/j.nbd.2019.104635_bb0165","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1212\/WNL.28.7.703","article-title":"Autosomal dominant system degeneration in Portuguese families of the Azores Islands. A new genetic disorder involving cerebellar, pyramidal, extrapyramidal and spinal cord motor functions","volume":"28","author":"Coutinho","year":"1978","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2019.104635_bb0170","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1038\/nn.3668","article-title":"Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors","volume":"17","author":"Crotti","year":"2014","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0175","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/j.neuroscience.2015.01.003","article-title":"Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1","volume":"289","author":"Cvetanovic","year":"2015","journal-title":"Neuroscience"},{"key":"10.1016\/j.nbd.2019.104635_bb0180","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1111\/j.1552-6569.2008.00260.x","article-title":"Axonal dysfunction in the deep white matter in Machado-Joseph disease","volume":"19","author":"D'Abreu","year":"2009","journal-title":"J. Neuroimaging"},{"key":"10.1016\/j.nbd.2019.104635_bb0185","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/S1474-4422(18)30042-5","article-title":"Survival in patients with spinocerebellar ataxia types 1, 2, 3, and 6 (EUROSCA): a longitudinal cohort study","volume":"17","author":"Diallo","year":"2018","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0190","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1515\/BC.2009.035","article-title":"Central nervous system: cholesterol turnover, brain development and neurodegeneration","volume":"390","author":"Dietschy","year":"2009","journal-title":"Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0195","doi-asserted-by":"crossref","first-page":"6469","DOI":"10.1128\/MCB.23.18.6469-6483.2003","article-title":"Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis","volume":"23","author":"Doss-Pepe","year":"2003","journal-title":"Mol. Cell Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0200","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1007\/978-3-319-71779-1_19","article-title":"Pharmacological therapies for Machado-Joseph disease","volume":"1049","author":"Duarte-Silva","year":"2018","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0205","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/j.neuroscience.2015.11.030","article-title":"Combined therapy with m-TOR-dependent and -independent autophagy inducers causes neurotoxicity in a mouse model of Machado-Joseph disease","volume":"313","author":"Duarte-Silva","year":"2016","journal-title":"Neuroscience"},{"key":"10.1016\/j.nbd.2019.104635_bb0210","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1093\/hmg\/ddq452","article-title":"The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability","volume":"20","author":"Durcan","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0215","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1016\/S1474-4422(10)70183-6","article-title":"Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond","volume":"9","author":"Durr","year":"2010","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0220","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1002\/ana.410390411","article-title":"Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features","volume":"39","author":"Durr","year":"1996","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0225","doi-asserted-by":"crossref","first-page":"5389","DOI":"10.1523\/JNEUROSCI.21-15-05389.2001","article-title":"Inflammatory genes are upregulated in expanded ataxin-3-expressing cell lines and spinocerebellar ataxia type 3 brains","volume":"21","author":"Evert","year":"2001","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0230","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1093\/jnen\/62.10.1006","article-title":"Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3","volume":"62","author":"Evert","year":"2003","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0235","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1007\/s12311-014-0619-7","article-title":"Spinal cord damage in Machado-Joseph disease","volume":"14","author":"Fahl","year":"2015","journal-title":"Cerebellum"},{"key":"10.1016\/j.nbd.2019.104635_bb0240","doi-asserted-by":"crossref","first-page":"1200","DOI":"10.1007\/s12035-013-8591-7","article-title":"Mutations of the Huntington\u2019s disease protein impact on the ATM-dependent signaling and repair pathways of the radiation-induced DNA double-strand breaks: corrective effect of statins and bisphosphonates","volume":"49","author":"Ferlazzo","year":"2014","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.nbd.2019.104635_bb0245","doi-asserted-by":"crossref","first-page":"9622","DOI":"10.1073\/pnas.1818042116","article-title":"Intrinsic mutant HTT-mediated defects in oligodendroglia cause myelination deficits and behavioral abnormalities in Huntington disease","volume":"116","author":"Ferrari Bardile","year":"2019","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"10.1016\/j.nbd.2019.104635_bb0250","doi-asserted-by":"crossref","first-page":"3017","DOI":"10.1016\/S0140-6736(16)31408-8","article-title":"Treatment of infantile-onset spinal muscular atrophy with nusinersen: a phase 2, open-label, dose-escalation study","volume":"388","author":"Finkel","year":"2016","journal-title":"Lancet"},{"key":"10.1016\/j.nbd.2019.104635_bb0255","doi-asserted-by":"crossref","first-page":"1723","DOI":"10.1056\/NEJMoa1702752","article-title":"Nusinersen versus sham control in infantile-onset spinal muscular atrophy","volume":"377","author":"Finkel","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.nbd.2019.104635_bb0260","doi-asserted-by":"crossref","first-page":"9030","DOI":"10.1038\/sj.onc.1207116","article-title":"Control of apoptosis by p53","volume":"22","author":"Fridman","year":"2003","journal-title":"Oncogene"},{"key":"10.1016\/j.nbd.2019.104635_bb0265","doi-asserted-by":"crossref","first-page":"16269","DOI":"10.1523\/JNEUROSCI.4000-11.2011","article-title":"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","volume":"31","author":"Furrer","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0270","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1004834","article-title":"Inactivation of PNKP by mutant ATXN3 triggers apoptosis by activating the DNA damage-response pathway in SCA3","volume":"11","author":"Gao","year":"2015","journal-title":"PLoS Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0275","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.42988","article-title":"Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription","volume":"8","author":"Gao","year":"2019","journal-title":"Elife"},{"issue":"6","key":"10.1016\/j.nbd.2019.104635_bb0280","doi-asserted-by":"crossref","first-page":"650","DOI":"10.1001\/jamaneurol.2019.0423","article-title":"Prevalence of carriers of intermediate and pathological polyglutamine disease-associated alleles among large population-based cohorts","volume":"76","author":"Gardiner","year":"2019","journal-title":"JAMA Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0285","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1086\/318184","article-title":"Ancestral origins of the Machado-Joseph disease mutation: a worldwide haplotype study","volume":"68","author":"Gaspar","year":"2001","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0290","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1038\/ng0793-295","article-title":"Chromosomal assignment of the second locus for autosomal dominant cerebellar ataxia (SCA2) to chromosome 12q23-24.1","volume":"4","author":"Gispert","year":"1993","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0295","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1038\/nrm.2017.73","article-title":"Regulation of heat shock transcription factors and their roles in physiology and disease","volume":"19","author":"Gomez-Pastor","year":"2018","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0300","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/j.tibs.2018.02.010","article-title":"The cellular response to transcription-blocking dna damage","volume":"43","author":"Gregersen","year":"2018","journal-title":"Trends Biochem. Sci."},{"key":"10.1016\/j.nbd.2019.104635_bb0305","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1002\/mds.25451","article-title":"A multimodal evaluation of microstructural white matter damage in spinocerebellar ataxia type 3","volume":"28","author":"Guimaraes","year":"2013","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2019.104635_bb0310","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/0022-510X(95)90927-I","article-title":"Spinocerebellar ataxia, type 3 (SCA3) is genetically identical to Machado-Joseph disease (MJD)","volume":"132","author":"Haberhausen","year":"1995","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.nbd.2019.104635_bb0315","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1016\/j.scr.2016.07.004","article-title":"Induced pluripotent stem cell - derived neurons for the study of spinocerebellar ataxia type 3","volume":"17","author":"Hansen","year":"2016","journal-title":"Stem Cell Res."},{"key":"10.1016\/j.nbd.2019.104635_bb0320","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0013695","article-title":"Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties","volume":"5","author":"Harris","year":"2010","journal-title":"PloS One"},{"key":"10.1016\/j.nbd.2019.104635_bb0325","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1046\/j.1440-1819.2003.01102.x","article-title":"Immunohistochemical study of neuronal intranuclear and cytoplasmic inclusions in Machado-Joseph disease","volume":"57","author":"Hayashi","year":"2003","journal-title":"Psychiatry Clin. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0330","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1038\/s41593-018-0242-x","article-title":"Microglia in neurodegeneration","volume":"21","author":"Hickman","year":"2018","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0335","doi-asserted-by":"crossref","first-page":"1212","DOI":"10.1016\/j.neuron.2015.02.026","article-title":"Mutant huntingtin downregulates myelin regulatory factor-mediated myelin gene expression and affects mature oligodendrocytes","volume":"85","author":"Huang","year":"2015","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2019.104635_bb0340","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1007\/s100380170060","article-title":"The genomic structure and expression of MJD, the Machado-Joseph disease gene","volume":"46","author":"Ichikawa","year":"2001","journal-title":"J. Hum. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0345","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1016\/j.bbrc.2016.03.152","article-title":"A novel nuclear DnaJ protein, DNAJC8, can suppress the formation of spinocerebellar ataxia 3 polyglutamine aggregation in a J-domain independent manner","volume":"474","author":"Ito","year":"2016","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.nbd.2019.104635_bb0350","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1016\/j.dnarep.2013.04.015","article-title":"DNA repair mechanisms in dividing and non-dividing cells","volume":"12","author":"Iyama","year":"2013","journal-title":"DNA Repair (Amst)"},{"key":"10.1016\/j.nbd.2019.104635_bb0355","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1038\/nature08467","article-title":"The DNA-damage response in human biology and disease","volume":"461","author":"Jackson","year":"2009","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2019.104635_bb0360","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1002\/ana.25212","article-title":"Neurochemical abnormalities in premanifest and early spinocerebellar ataxias","volume":"83","author":"Joers","year":"2018","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0365","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1016\/S1474-4422(16)30350-7","article-title":"DNA repair in the trinucleotide repeat disorders","volume":"16","author":"Jones","year":"2017","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0370","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1007\/s00415-013-7186-6","article-title":"White matter damage is related to ataxia severity in SCA3","volume":"261","author":"Kang","year":"2014","journal-title":"J. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0375","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/ng1194-221","article-title":"CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1","volume":"8","author":"Kawaguchi","year":"1994","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0380","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/ng1194-221","article-title":"CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1","volume":"8","author":"Kawaguchi","year":"1994","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0385","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1159\/000339207","article-title":"Patterns of mitochondrial DNA damage in blood and brain tissues of a transgenic mouse model of Machado-Joseph disease","volume":"11","author":"Kazachkova","year":"2013","journal-title":"Neurodegener. Dis."},{"key":"10.1016\/j.nbd.2019.104635_bb0390","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1038\/s41572-019-0074-3","article-title":"Spinocerebellar ataxia","volume":"5","author":"Klockgether","year":"2019","journal-title":"Nat. Rev. Dis. Primers"},{"key":"10.1016\/j.nbd.2019.104635_bb0395","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1038\/nature10671","article-title":"Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease","volume":"480","author":"Koch","year":"2011","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2019.104635_bb0400","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1016\/S0962-8924(00)01852-3","article-title":"Aggresomes, inclusion bodies and protein aggregation","volume":"10","author":"Kopito","year":"2000","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0405","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1016\/j.neuron.2012.05.009","article-title":"Sustained therapeutic reversal of Huntington\u2019s disease by transient repression of huntingtin synthesis","volume":"74","author":"Kordasiewicz","year":"2012","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2019.104635_bb0410","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1007\/978-1-4939-7825-0_23","article-title":"Translating antisense technology into a treatment for huntington\u2019s disease","volume":"1780","author":"Lane","year":"2018","journal-title":"Methods Mol. Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0415","doi-asserted-by":"crossref","first-page":"45004","DOI":"10.1074\/jbc.M205259200","article-title":"Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities","volume":"277","author":"Li","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0420","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.pneurobio.2015.06.004","article-title":"Toward therapeutic targets for SCA3: insight into the role of Machado-Joseph disease protein ataxin-3 in misfolded proteins clearance","volume":"132","author":"Li","year":"2015","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.nbd.2019.104635_bb0425","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1016\/j.immuni.2017.06.006","article-title":"Reactive astrocytes: production, function, and therapeutic potential","volume":"46","author":"Liddelow","year":"2017","journal-title":"Immunity"},{"key":"10.1016\/j.nbd.2019.104635_bb0430","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1016\/S0167-4781(99)00004-4","article-title":"Conserved domains and lack of evidence for polyglutamine length polymorphism in the chicken homolog of the Machado-Joseph disease gene product ataxin-3","volume":"1444","author":"Linhartov\u00e1","year":"1999","journal-title":"Biochim. Biophys. Acta Gene Struct. Expr."},{"key":"10.1016\/j.nbd.2019.104635_bb0435","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1007\/s12311-015-0684-6","article-title":"Peripheral neuropathy in spinocerebellar ataxia type 1, 2, 3, and 6","volume":"15","author":"Linnemann","year":"2016","journal-title":"Cerebellum"},{"key":"10.1016\/j.nbd.2019.104635_bb0440","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pbio.2000733","article-title":"The Machado-Joseph disease deubiquitinase ataxin-3 regulates the stability and apoptotic function of p53","volume":"14","author":"Liu","year":"2016","journal-title":"PLoS Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0445","doi-asserted-by":"crossref","first-page":"2370","DOI":"10.1007\/s00415-013-6998-8","article-title":"Widespread neuronal damage and cognitive dysfunction in spinocerebellar ataxia type 3","volume":"260","author":"Lopes","year":"2013","journal-title":"J. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0450","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1016\/j.molcel.2005.11.025","article-title":"MDC1 maintains genomic stability by participating in the amplification of ATM-dependent DNA damage signals","volume":"21","author":"Lou","year":"2006","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2019.104635_bb0455","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1016\/j.neulet.2006.09.007","article-title":"Dissociation of grey and white matter reduction in spinocerebellar ataxia type 3 and 6: a voxel-based morphometry study","volume":"408","author":"Lukas","year":"2006","journal-title":"Neurosci. Lett."},{"key":"10.1016\/j.nbd.2019.104635_bb0460","first-page":"54","article-title":"Correlation between CAG repeat length and clinical features in Machado-Joseph disease","volume":"57","author":"Maciel","year":"1995","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0465","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1016\/j.neuron.2014.06.034","article-title":"DNA damage and its links to neurodegeneration","volume":"83","author":"Madabhushi","year":"2014","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2019.104635_bb0470","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1016\/j.cell.2007.09.040","article-title":"RNF8 ubiquitylates histones at DNA double-strand breaks and promotes assembly of repair proteins","volume":"131","author":"Mailand","year":"2007","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2019.104635_bb0475","doi-asserted-by":"crossref","DOI":"10.1007\/s13311-019-00768-7","article-title":"DNA damage repair in huntington\u2019s disease and other neurodegenerative diseases","author":"Maiuri","year":"2019","journal-title":"Neurotherapeutics"},{"key":"10.1016\/j.nbd.2019.104635_bb0480","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1093\/hmg\/ddy328","article-title":"Cordycepin activates autophagy through AMPK phosphorylation to reduce abnormalities in Machado-Joseph disease models","volume":"28","author":"Marcelo","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0485","doi-asserted-by":"crossref","DOI":"10.1101\/cshperspect.a012716","article-title":"DNA damage sensing by the ATM and ATR kinases","volume":"5","author":"Marechal","year":"2013","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0490","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.15252\/embr.201439225","article-title":"Cholesterol in brain disease: sometimes determinant and frequently implicated","volume":"15","author":"Martin","year":"2014","journal-title":"EMBO Rep."},{"key":"10.1016\/j.nbd.2019.104635_bb0495","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1093\/hmg\/4.5.807","article-title":"Molecular features of the CAG repeats and clinical manifestation of Machado-Joseph disease","volume":"4","author":"Maruyama","year":"1995","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0500","doi-asserted-by":"crossref","DOI":"10.1242\/dmm.031930","article-title":"The central role of DNA damage and repair in CAG repeat diseases","volume":"11","author":"Massey","year":"2018","journal-title":"Dis. Model Mech."},{"key":"10.1016\/j.nbd.2019.104635_bb0505","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1111\/jnc.14541","article-title":"Machado-Joseph disease\/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapy","volume":"148","author":"Matos","year":"2018","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2019.104635_bb0510","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1038\/sj.emboj.7600081","article-title":"Molecular clearance of ataxin-3 is regulated by a mammalian E4","volume":"23","author":"Matsumoto","year":"2004","journal-title":"EMBO J."},{"key":"10.1016\/j.nbd.2019.104635_bb0515","doi-asserted-by":"crossref","first-page":"25485","DOI":"10.1074\/jbc.M803111200","article-title":"Cleavage-mediated activation of Chk1 during apoptosis","volume":"283","author":"Matsuura","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0520","doi-asserted-by":"crossref","first-page":"1354","DOI":"10.1126\/science.294.5545.1354","article-title":"CNS synaptogenesis promoted by glia-derived cholesterol","volume":"294","author":"Mauch","year":"2001","journal-title":"Science"},{"key":"10.1016\/j.nbd.2019.104635_bb0525","doi-asserted-by":"crossref","first-page":"2485","DOI":"10.1016\/j.biocel.2009.08.003","article-title":"Proteomic and biochemical analyses unveil tight interaction of ataxin-3 with tubulin","volume":"41","author":"Mazzucchelli","year":"2009","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0530","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1002\/ana.25264","article-title":"Oligonucleotide therapy mitigates disease in Spinocerebellar Ataxia Type 3 mice","volume":"81","author":"McLoughlin","year":"2018","journal-title":"Ann.Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0535","doi-asserted-by":"crossref","first-page":"818","DOI":"10.1038\/s41419-018-0848-7","article-title":"Activation of the DNA damage response in vivo in synucleinopathy models of Parkinson\u2019s disease","volume":"9","author":"Milanese","year":"2018","journal-title":"Cell Death Dis."},{"key":"10.1016\/j.nbd.2019.104635_bb0540","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1016\/j.omtn.2017.04.005","article-title":"Evaluation of antisense oligonucleotides targeting atxn3 in sca3 mouse models","volume":"7","author":"Moore","year":"2017","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"10.1016\/j.nbd.2019.104635_bb0545","doi-asserted-by":"crossref","DOI":"10.1016\/j.scr.2019.101504","article-title":"Antisense oligonucleotide therapy rescues aggresome formation in a novel Spinocerebellar Ataxia type 3 human embryonic stem cell line","volume":"39","author":"Moore","year":"2019","journal-title":"Stem Cell Res."},{"key":"10.1016\/j.nbd.2019.104635_bb0550","doi-asserted-by":"crossref","first-page":"3334","DOI":"10.1093\/hmg\/ddp274","article-title":"CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3","volume":"18","author":"Mueller","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0555","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0052396","article-title":"Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice","volume":"8","author":"Nobrega","year":"2013","journal-title":"PloS One"},{"issue":"5","key":"10.1016\/j.nbd.2019.104635_bb0560","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1007\/s00401-019-02019-7","article-title":"Restoring brain cholesterol turnover improves autophagy and has therapeutic potential in mouse models of spinocerebellar ataxia","volume":"138","author":"Nobrega","year":"2019","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.nbd.2019.104635_bb0565","doi-asserted-by":"crossref","first-page":"47","DOI":"10.2174\/092986708783330692","article-title":"Aggresome formation and neurodegenerative diseases: therapeutic implications","volume":"15","author":"Olzmann","year":"2008","journal-title":"Curr. Med. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0570","doi-asserted-by":"crossref","DOI":"10.1038\/srep28220","article-title":"Fibroblasts of Machado Joseph disease patients reveal autophagy impairment","volume":"6","author":"Onofre","year":"2016","journal-title":"Sci. Rep."},{"key":"10.1016\/j.nbd.2019.104635_bb0575","doi-asserted-by":"crossref","DOI":"10.1155\/2012\/292598","article-title":"Cholesterol: its regulation and role in central nervous system disorders","volume":"2012","author":"Orth","year":"2012","journal-title":"Cholesterol"},{"key":"10.1016\/j.nbd.2019.104635_bb0580","doi-asserted-by":"crossref","DOI":"10.1155\/2016\/6701793","article-title":"Autophagy promoted the degradation of mutant atxn3 in neurally differentiated spinocerebellar ataxia-3 human induced pluripotent stem cells","volume":"2016","author":"Ou","year":"2016","journal-title":"Biomed. Res. Int."},{"key":"10.1016\/j.nbd.2019.104635_bb0585","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1089\/scd.2017.0209","article-title":"Crispr\/cas9-targeted deletion of polyglutamine in spinocerebellar ataxia type 3-derived induced pluripotent stem cells","volume":"27","author":"Ouyang","year":"2018","journal-title":"Stem Cells Dev."},{"key":"10.1016\/j.nbd.2019.104635_bb0590","doi-asserted-by":"crossref","first-page":"2274","DOI":"10.1002\/glia.22892","article-title":"CCL11 enhances excitotoxic neuronal death by producing reactive oxygen species in microglia","volume":"63","author":"Parajuli","year":"2015","journal-title":"Glia"},{"key":"10.1016\/j.nbd.2019.104635_bb0595","doi-asserted-by":"crossref","DOI":"10.1155\/2015\/125273","article-title":"Parkinsonism in spinocerebellar ataxia","volume":"2015","author":"Park","year":"2015","journal-title":"Biomed. Res. Int."},{"key":"10.1016\/j.nbd.2019.104635_bb0600","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1055\/s-2007-971172","article-title":"Dominantly inherited ataxias: lessons learned from Machado-Joseph disease\/spinocerebellar ataxia type 3","volume":"27","author":"Paulson","year":"2007","journal-title":"Semin Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0605","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1002\/ana.410410408","article-title":"Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain","volume":"41","author":"Paulson","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0610","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1016\/S0896-6273(00)80943-5","article-title":"Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3","volume":"19","author":"Paulson","year":"1997","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2019.104635_bb0615","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1038\/nrn.2017.92","article-title":"Polyglutamine spinocerebellar ataxias - from genes to potential treatments","volume":"18","author":"Paulson","year":"2017","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0620","doi-asserted-by":"crossref","DOI":"10.1038\/ncomms13821","article-title":"CCT complex restricts neuropathogenic protein aggregation via autophagy","volume":"7","author":"Pavel","year":"2016","journal-title":"Nat. Commun."},{"key":"10.1016\/j.nbd.2019.104635_bb0625","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1007\/s12311-011-0252-7","article-title":"Sleep disorders in machado-joseph disease: frequency, discriminative thresholds, predictive values, and correlation with ataxia-related motor and non-motor features","volume":"10","author":"Pedroso","year":"2011","journal-title":"Cerebellum"},{"key":"10.1016\/j.nbd.2019.104635_bb0630","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.15252\/embj.201695151","article-title":"Ataxin-3 consolidates the MDC1-dependent DNA double-strand break response by counteracting the SUMO-targeted ubiquitin ligase RNF4","volume":"36","author":"Pfeiffer","year":"2017","journal-title":"EMBO J."},{"key":"10.1016\/j.nbd.2019.104635_bb0635","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/j.nbd.2015.08.011","article-title":"Increased central microglial activation associated with peripheral cytokine levels in premanifest Huntington\u2019s disease gene carriers","volume":"83","author":"Politis","year":"2015","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2019.104635_bb0640","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1186\/s12974-017-0880-z","article-title":"Inhibition of colony-stimulating factor 1 receptor early in disease ameliorates motor deficits in SCA1 mice","volume":"14","author":"Qu","year":"2017","journal-title":"J. Neuroinflammation"},{"key":"10.1016\/j.nbd.2019.104635_bb0645","doi-asserted-by":"crossref","first-page":"3362","DOI":"10.1093\/hmg\/ddx224","article-title":"Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes","volume":"26","author":"Ramani","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0650","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1093\/hmg\/ddp482","article-title":"Proteotoxic stress increases nuclear localization of ataxin-3","volume":"19","author":"Reina","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0655","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1146\/annurev.biochem.78.082307.091526","article-title":"Regulation and cellular roles of ubiquitin-specific deubiquitinating enzymes","volume":"78","author":"Reyes-Turcu","year":"2009","journal-title":"Ann. Rev. Biochem."},{"key":"10.1016\/j.nbd.2019.104635_bb0660","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1002\/ana.25297","article-title":"Structural signature of SCA3: from presymptomatic to late disease stages","volume":"84","author":"Rezende","year":"2018","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0665","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.scr.2018.05.012","article-title":"Derivation of an induced pluripotent stem cell line (MUSIi004-A) from dermal fibroblasts of a 48-year-old spinocerebellar ataxia type 3 patient","volume":"30","author":"Ritthaphai","year":"2018","journal-title":"Stem Cell Res."},{"key":"10.1016\/j.nbd.2019.104635_bb0670","doi-asserted-by":"crossref","first-page":"1126","DOI":"10.1096\/fj.06-7002com","article-title":"Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado-Joseph disease protein ataxin-3","volume":"21","author":"Rodrigues","year":"2007","journal-title":"FASEB J."},{"key":"10.1016\/j.nbd.2019.104635_bb0675","doi-asserted-by":"crossref","first-page":"1154","DOI":"10.1016\/j.bbamcr.2010.07.004","article-title":"Absence of ataxin-3 leads to cytoskeletal disorganization and increased cell death","volume":"1803","author":"Rodrigues","year":"2010","journal-title":"Biochim. Biophys. Acta."},{"key":"10.1016\/j.nbd.2019.104635_bb0680","doi-asserted-by":"crossref","first-page":"1909","DOI":"10.1038\/mt.2013.152","article-title":"Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice","volume":"21","author":"Rodriguez-Lebron","year":"2013","journal-title":"Mol. Ther."},{"key":"10.1016\/j.nbd.2019.104635_bb0685","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1186\/s13023-016-0543-7","article-title":"Ataxia telangiectasia: a review","volume":"11","author":"Rothblum-Oviatt","year":"2016","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.nbd.2019.104635_bb0690","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/S0006-8993(02)03278-X","article-title":"Degeneration of the external cuneate nucleus in spinocerebellar ataxia type 3 (Machado-Joseph disease)","volume":"953","author":"Rub","year":"2002","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2019.104635_bb0695","doi-asserted-by":"crossref","first-page":"2115","DOI":"10.1093\/brain\/awf208","article-title":"Spinocerebellar ataxia type 3 (Machado\u2013Joseph disease): severe destruction of the lateral reticular nucleus","volume":"125","author":"R\u00fcb","year":"2002","journal-title":"Brain"},{"key":"10.1016\/j.nbd.2019.104635_bb0700","doi-asserted-by":"crossref","first-page":"2257","DOI":"10.1093\/brain\/awg234","article-title":"\u2019Thalamic involvement in a spinocerebellar ataxia type 2 (SCA2) and a spinocerebellar ataxia type 3 (SCA3) patient, and its clinical relevance","volume":"126","author":"Rub","year":"2003","journal-title":"Brain"},{"key":"10.1016\/j.nbd.2019.104635_bb0705","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1111\/j.1365-2990.2004.00554.x","article-title":"Degeneration of the central vestibular system in spinocerebellar ataxia type 3 (SCA3) patients and its possible clinical significance","volume":"30","author":"Rub","year":"2004","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.nbd.2019.104635_bb0710","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1097\/WCO.0b013e3282f7673d","article-title":"New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado-Joseph disease)","volume":"21","author":"R\u00fcb","year":"2008","journal-title":"Curr. Opin. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0715","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1016\/j.pneurobio.2013.01.001","article-title":"Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7","volume":"104","author":"Rub","year":"2013","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.nbd.2019.104635_bb0720","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/j.nbd.2010.08.023","article-title":"17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy","volume":"41","author":"Rusmini","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2019.104635_bb0725","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1038\/nn1426","article-title":"High cholesterol level is essential for myelin membrane growth","volume":"8","author":"Saher","year":"2005","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0730","doi-asserted-by":"crossref","first-page":"25571","DOI":"10.1074\/jbc.M115.676247","article-title":"The social amoeba dictyostelium discoideum is highly resistant to polyglutamine aggregation","volume":"290","author":"Santarriaga","year":"2015","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0735","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1016\/j.molcel.2018.07.008","article-title":"SRCP1 conveys resistance to polyglutamine aggregation","volume":"71","author":"Santarriaga","year":"2018","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2019.104635_bb0740","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/j.molcel.2011.05.036","article-title":"Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP","volume":"43","author":"Scaglione","year":"2011","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2019.104635_bb0745","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1007\/s12311-011-0340-8","article-title":"Pathoanatomy of cerebellar degeneration in spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3)","volume":"11","author":"Scherzed","year":"2012","journal-title":"Cerebellum"},{"key":"10.1016\/j.nbd.2019.104635_bb0750","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1111\/j.1750-3639.1998.tb00193.x","article-title":"An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients","volume":"8","author":"Schmidt","year":"1998","journal-title":"Brain Pathol."},{"key":"10.1016\/j.nbd.2019.104635_bb0755","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1002\/ana.10101","article-title":"Protein surveillance machinery in brains with spinocerebellar ataxia type 3: redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions","volume":"51","author":"Schmidt","year":"2002","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2019.104635_bb0760","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1007\/s100480050015","article-title":"Characterization of the rat spinocerebellar ataxia type 3 gene","volume":"1","author":"Schmitt","year":"1997","journal-title":"Neurogenetics"},{"key":"10.1016\/j.nbd.2019.104635_bb0765","doi-asserted-by":"crossref","first-page":"734","DOI":"10.1016\/j.bbrc.2007.08.062","article-title":"Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination","volume":"362","author":"Schmitt","year":"2007","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.nbd.2019.104635_bb0770","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1007\/s00401-010-0717-7","article-title":"Axonal inclusions in spinocerebellar ataxia type 3","volume":"120","author":"Seidel","year":"2010","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.nbd.2019.104635_bb0775","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1111\/j.1365-2990.2011.01198.x","article-title":"The HSPB8-BAG3 chaperone complex is upregulated in astrocytes in the human brain affected by protein aggregation diseases","volume":"38","author":"Seidel","year":"2012","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.nbd.2019.104635_bb0780","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1111\/bpa.12412","article-title":"On the distribution of intranuclear and cytoplasmic aggregates in the brainstem of patients with spinocerebellar ataxia type 2 and 3","volume":"27","author":"Seidel","year":"2017","journal-title":"Brain Pathol."},{"key":"10.1016\/j.nbd.2019.104635_bb0785","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1159\/000484516","article-title":"Assessment of sleep spindle density among genetically positive spinocerebellar ataxias types 1, 2, and 3 patients","volume":"25","author":"Seshagiri","year":"2018","journal-title":"Ann. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0790","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1186\/s40478-019-0723-5","article-title":"Early neuronal accumulation of DNA double strand breaks in Alzheimer\u2019s disease","volume":"7","author":"Shanbhag","year":"2019","journal-title":"Acta Neuropathol. Commun."},{"key":"10.1016\/j.nbd.2019.104635_bb0795","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1038\/nrm3546","article-title":"The ATM protein kinase: regulating the cellular response to genotoxic stress, and more","volume":"14","author":"Shiloh","year":"2013","journal-title":"Nat. Rev. Mol Cell. Biol."},{"key":"10.1016\/j.nbd.2019.104635_bb0800","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1007\/s12311-015-0719-z","article-title":"Cytokines in Machado Joseph disease\/spinocerebellar Ataxia 3","volume":"15","author":"da Silva Carvalho","year":"2016","journal-title":"Cerebellum"},{"key":"10.1016\/j.nbd.2019.104635_bb0805","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.nbd.2010.05.021","article-title":"Motor uncoordination and neuropathology in a transgenic mouse model of Machado-Joseph disease lacking intranuclear inclusions and ataxin-3 cleavage products","volume":"40","author":"Silva-Fernandes","year":"2010","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2019.104635_bb0810","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1007\/s13311-013-0255-9","article-title":"Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease","volume":"11","author":"Silva-Fernandes","year":"2014","journal-title":"Neurotherapeutics"},{"key":"10.1016\/j.nbd.2019.104635_bb0815","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1111\/neup.12433","article-title":"Deregulation of autophagy in postmortem brains of Machado-Joseph disease patients","volume":"38","author":"Sittler","year":"2018","journal-title":"Neuropathology"},{"key":"10.1016\/j.nbd.2019.104635_bb0820","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/B978-0-12-380888-2.00003-0","article-title":"The ATM-Chk2 and ATR-Chk1 pathways in DNA damage signaling and cancer","volume":"108","author":"Smith","year":"2010","journal-title":"Adv. Cancer Res."},{"key":"10.1016\/j.nbd.2019.104635_bb0825","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1136\/jnnp.60.3.354-a","article-title":"Homozygosity for Machado-Joseph disease gene enhances phenotypic severity","volume":"60","author":"Sobue","year":"1996","journal-title":"J. Neurol. Neurosurg.Psychiatry"},{"key":"10.1016\/j.nbd.2019.104635_bb0830","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1016\/j.tins.2009.08.002","article-title":"Molecular dissection of reactive astrogliosis and glial scar formation","volume":"32","author":"Sofroniew","year":"2009","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0835","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.1716071115","article-title":"Karyopherin alpha-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3","volume":"115","author":"Sowa","year":"2018","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"10.1016\/j.nbd.2019.104635_bb0840","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1111\/nan.12055","article-title":"Schwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3","volume":"40","author":"Suga","year":"2014","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.nbd.2019.104635_bb0845","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1016\/j.nbd.2014.09.020","article-title":"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","volume":"73","author":"Switonski","year":"2015","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2019.104635_bb0850","doi-asserted-by":"crossref","first-page":"2307","DOI":"10.1056\/NEJMoa1900907","article-title":"Targeting huntingtin expression in patients with huntington\u2019s disease","volume":"380","author":"Tabrizi","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.nbd.2019.104635_bb0855","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1038\/ng0793-300","article-title":"The gene for Machado-Joseph disease maps to human chromosome 14q","volume":"4","author":"Takiyama","year":"1993","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0860","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1093\/emboj\/19.1.94","article-title":"Recognition of the polyubiquitin proteolytic signal","volume":"19","author":"Thrower","year":"2000","journal-title":"EMBO J."},{"key":"10.1016\/j.nbd.2019.104635_bb0865","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1038\/emboj.2008.289","article-title":"Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3","volume":"28","author":"Todi","year":"2009","journal-title":"EMBO J."},{"key":"10.1016\/j.nbd.2019.104635_bb0870","doi-asserted-by":"crossref","first-page":"39303","DOI":"10.1074\/jbc.M110.181610","article-title":"Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117","volume":"285","author":"Todi","year":"2010","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0875","doi-asserted-by":"crossref","DOI":"10.1038\/srep35200","article-title":"Antisense oligonucleotide-mediated exon skipping as a strategy to reduce proteolytic cleavage of ataxin-3","volume":"6","author":"Toonen","year":"2016","journal-title":"Sci. Rep."},{"key":"10.1016\/j.nbd.2019.104635_bb0880","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1016\/j.omtn.2017.06.019","article-title":"Antisense oligonucleotide-mediated removal of the polyglutamine repeat in spinocerebellar ataxia type 3 mice","volume":"8","author":"Toonen","year":"2017","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"10.1016\/j.nbd.2019.104635_bb0885","doi-asserted-by":"crossref","DOI":"10.1186\/s13024-018-0261-9","article-title":"Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model","volume":"13","author":"Toonen","year":"2018","journal-title":"Mol. Neurodegener."},{"key":"10.1016\/j.nbd.2019.104635_bb0890","doi-asserted-by":"crossref","first-page":"4532","DOI":"10.1093\/nar\/gkx095","article-title":"Ataxin-3 promotes genome integrity by stabilizing Chk1","volume":"45","author":"Tu","year":"2017","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2019.104635_bb0895","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1007\/s004010100364","article-title":"Non-expanded polyglutamine proteins in intranuclear inclusions of hereditary ataxias \u2013 triple-labeling immunofluorescence study","volume":"102","author":"Uchihara","year":"2001","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.nbd.2019.104635_bb0900","first-page":"746","article-title":"Dysregulation of cholesterol balance in the brain: contribution to neurodegenerative diseases","volume":"5","author":"Vance","year":"2012","journal-title":"Dis. Model Mech."},{"key":"10.1016\/j.nbd.2019.104635_bb0905","doi-asserted-by":"crossref","first-page":"4677","DOI":"10.1093\/hmg\/ddq398","article-title":"HSPB7 is the most potent polyQ aggregation suppressor within the HSPB family of molecular chaperones","volume":"19","author":"Vos","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0910","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1016\/j.neuroscience.2017.11.051","article-title":"Experimental and clinical strategies for treating spinocerebellar ataxia type 3","volume":"371","author":"Wang","year":"2018","journal-title":"Neuroscience"},{"key":"10.1016\/j.nbd.2019.104635_bb0915","doi-asserted-by":"crossref","first-page":"28576","DOI":"10.1074\/jbc.M111.299990","article-title":"Ataxin-3 regulates aggresome formation of copper-zinc superoxide dismutase (SOD1) by editing K63-linked polyubiquitin chains","volume":"287","author":"Wang","year":"2012","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0920","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1080\/15548627.2016.1245262","article-title":"Autophagy substrate SQSTM1\/p62 regulates chromatin ubiquitination during the DNA damage response","volume":"13","author":"Wang","year":"2017","journal-title":"Autophagy"},{"key":"10.1016\/j.nbd.2019.104635_bb0925","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1038\/70532","article-title":"Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70","volume":"23","author":"Warrick","year":"1999","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0930","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.molcel.2005.02.030","article-title":"Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism","volume":"18","author":"Warrick","year":"2005","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2019.104635_bb0935","doi-asserted-by":"crossref","first-page":"7782","DOI":"10.1523\/JNEUROSCI.1142-17.2017","article-title":"Calpain inhibition is protective in machado-joseph disease zebrafish due to induction of autophagy","volume":"37","author":"Watchon","year":"2017","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0940","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1074\/jbc.RA118.005801","article-title":"Physiological and pathophysiological characteristics of ataxin-3 isoforms","volume":"294","author":"Weishaupl","year":"2019","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0945","doi-asserted-by":"crossref","first-page":"26436","DOI":"10.1074\/jbc.M803692200","article-title":"The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains","volume":"283","author":"Winborn","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0950","first-page":"487","article-title":"Mutant huntingtin reduction in astrocytes slows disease progression in the BACHD conditional Huntington\u2019s disease mouse model","volume":"28","author":"Wood","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2019.104635_bb0955","doi-asserted-by":"crossref","first-page":"9985","DOI":"10.1074\/jbc.RA118.007117","article-title":"The N terminus of the small heat shock protein HSPB7 drives its polyQ aggregation-suppressing activity","volume":"294","author":"Wu","year":"2019","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2019.104635_bb0960","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/j.dnarep.2018.08.004","article-title":"Mutations, protein homeostasis, and epigenetic control of genome integrity","volume":"71","author":"Xie","year":"2018","journal-title":"DNA Repair (Amst)"},{"key":"10.1016\/j.nbd.2019.104635_bb0965","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1007\/s004010000277","article-title":"Involvement of the cerebral cortex and autonomic ganglia in Machado-Joseph disease","volume":"101","author":"Yamada","year":"2001","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.nbd.2019.104635_bb0970","doi-asserted-by":"crossref","first-page":"9101","DOI":"10.1523\/JNEUROSCI.0111-17.2017","article-title":"Synergistic toxicity of polyglutamine-expanded tata-binding protein in glia and neuronal cells: therapeutic implications for spinocerebellar ataxia 17","volume":"37","author":"Yang","year":"2017","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2019.104635_bb0975","article-title":"SUMO-1 modification on K166 of polyQ-expanded ataxin-3 strengthens its stability and increases its cytotoxicity","volume":"8","author":"Zhou","year":"2013","journal-title":"PloS One"},{"key":"10.1016\/j.nbd.2019.104635_bb0980","doi-asserted-by":"crossref","first-page":"14997","DOI":"10.3390\/ijms150914997","article-title":"p62\/sequestosome 1 regulates aggresome formation of pathogenic ataxin-3 with expanded polyglutamine","volume":"15","author":"Zhou","year":"2014","journal-title":"Int. J. Mol. Sci."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996119303109?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996119303109?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T08:54:18Z","timestamp":1778576058000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996119303109"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,2]]},"references-count":196,"alternative-id":["S0969996119303109"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2019.104635","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2020,2]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Pathogenesis of SCA3 and implications for other polyglutamine diseases","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2019.104635","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2019 Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"104635"}}