{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T10:20:42Z","timestamp":1776853242314,"version":"3.51.2"},"reference-count":75,"publisher":"Oxford University Press (OUP)","issue":"15","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2011,8,1]]},"DOI":"10.1093\/hmg\/ddr203","type":"journal-article","created":{"date-parts":[[2011,5,6]],"date-time":"2011-05-06T00:35:56Z","timestamp":1304642156000},"page":"2996-3009","source":"Crossref","is-referenced-by-count":95,"title":["Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans : rescue by the DAF-16 and HSF-1 pathways"],"prefix":"10.1093","volume":"20","author":[{"given":"Andreia","family":"Teixeira-Castro","sequence":"first","affiliation":[]},{"given":"Michael","family":"Ailion","sequence":"additional","affiliation":[]},{"given":"Ana","family":"Jalles","sequence":"additional","affiliation":[]},{"given":"Heather R.","family":"Brignull","sequence":"additional","affiliation":[]},{"given":"Jo\u00e3o L.","family":"Vila\u00e7a","sequence":"additional","affiliation":[]},{"given":"Nuno","family":"Dias","sequence":"additional","affiliation":[]},{"given":"Pedro","family":"Rodrigues","sequence":"additional","affiliation":[]},{"given":"Jo\u00e3o F.","family":"Oliveira","sequence":"additional","affiliation":[]},{"given":"Andreia","family":"Neves-Carvalho","sequence":"additional","affiliation":[]},{"given":"Richard I.","family":"Morimoto","sequence":"additional","affiliation":[]},{"given":"Patr\u00edcia","family":"Maciel","sequence":"additional","affiliation":[]}],"member":"286","published-online":{"date-parts":[[2011,5,5]]},"reference":[{"key":"key\n\t\t\t\t20170522142141_DDR203C1","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1136\/jnnp.59.4.449","article-title":"Machado\u2014Joseph disease mutations as the genetic basis of most spinocerebellar ataxias in Germany","volume":"59","author":"Schols","year":"1995","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"key\n\t\t\t\t20170522142141_DDR203C2","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/S1474-4422(04)00737-9","article-title":"Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis","volume":"3","author":"Schols","year":"2004","journal-title":"Lancet Neurol."},{"key":"key\n\t\t\t\t20170522142141_DDR203C3","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":"key\n\t\t\t\t20170522142141_DDR203C4","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1038\/nrg1691","article-title":"Diseases of unstable repeat expansion: mechanisms and common principles","volume":"6","author":"Gatchel","year":"2005","journal-title":"Nat. Rev. Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C5","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/S0968-0004(98)01350-4","article-title":"Glutamine repeats and neurodegenerative diseases: molecular aspects","volume":"24","author":"Perutz","year":"1999","journal-title":"Trends Biochem. Sci."},{"key":"key\n\t\t\t\t20170522142141_DDR203C6","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/0896-6273(95)90138-8","article-title":"When more is less: pathogenesis of glutamine repeat neurodegenerative diseases","volume":"15","author":"Ross","year":"1995","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170522142141_DDR203C7","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/j.ygeno.2004.02.012","article-title":"Genomic structure, promoter activity, and developmental expression of the mouse homologue of the Machado\u2014Joseph disease (MJD) gene","volume":"84","author":"do Carmo Costa","year":"2004","journal-title":"Genomics"},{"key":"key\n\t\t\t\t20170522142141_DDR203C8","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\u2014Joseph disease protein ataxin-3","volume":"21","author":"Rodrigues","year":"2007","journal-title":"FASEB J."},{"key":"key\n\t\t\t\t20170522142141_DDR203C9","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":"key\n\t\t\t\t20170522142141_DDR203C10","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":"key\n\t\t\t\t20170522142141_DDR203C11","doi-asserted-by":"crossref","first-page":"8892","DOI":"10.1073\/pnas.1530212100","article-title":"Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates","volume":"100","author":"Donaldson","year":"2003","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522142141_DDR203C12","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1016\/j.bbrc.2004.07.131","article-title":"Structural and functional analysis of the Josephin domain of the polyglutamine protein ataxin-3","volume":"322","author":"Chow","year":"2004","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"key\n\t\t\t\t20170522142141_DDR203C13","doi-asserted-by":"crossref","first-page":"1619","DOI":"10.1016\/j.bbamcr.2007.07.012","article-title":"NEDD8: a new ataxin-3 interactor","volume":"1773","author":"Ferro","year":"2007","journal-title":"Biochim. Biophys. Acta."},{"key":"key\n\t\t\t\t20170522142141_DDR203C14","first-page":"139","article-title":"Epidemiology and clinical aspects of Machado\u2014Joseph disease","volume":"61","author":"Sequeiros","year":"1993","journal-title":"Adv. Neurol."},{"key":"key\n\t\t\t\t20170522142141_DDR203C15","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1007\/s12311-008-0013-4","article-title":"SCA3: neurological features, pathogenesis and animal models","volume":"7","author":"Riess","year":"2008","journal-title":"Cerebellum"},{"key":"key\n\t\t\t\t20170522142141_DDR203C16","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1038\/nature05292","article-title":"Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases","volume":"443","author":"Lin","year":"2006","journal-title":"Nature"},{"key":"key\n\t\t\t\t20170522142141_DDR203C17","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1097\/00019052-199408000-00001","article-title":"Aging as a major risk for degenerative diseases of the central nervous system","volume":"7","author":"Amaducci","year":"1994","journal-title":"Curr. Opin. Neurol."},{"key":"key\n\t\t\t\t20170522142141_DDR203C18","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1038\/nrn2474","article-title":"The insulin paradox: aging, proteotoxicity and neurodegeneration","volume":"9","author":"Cohen","year":"2008","journal-title":"Nat. Rev. Neurosci."},{"key":"key\n\t\t\t\t20170522142141_DDR203C19","doi-asserted-by":"crossref","first-page":"14914","DOI":"10.1073\/pnas.0902882106","article-title":"Collapse of proteostasis represents an early molecular event in Caenorhabditis elegans aging","volume":"106","author":"Ben-Zvi","year":"2009","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522142141_DDR203C20","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.1126\/science.1083701","article-title":"Regulation of aging and age-related disease by DAF-16 and heat-shock factor","volume":"300","author":"Hsu","year":"2003","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522142141_DDR203C21","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1091\/mbc.E03-07-0532","article-title":"Regulation of longevity in Caenorhabditis elegans by heat shock factor and molecular chaperones","volume":"15","author":"Morley","year":"2004","journal-title":"Mol. Biol. Cell"},{"key":"key\n\t\t\t\t20170522142141_DDR203C22","doi-asserted-by":"crossref","first-page":"1604","DOI":"10.1126\/science.1124646","article-title":"Opposing activities protect against age-onset proteotoxicity","volume":"313","author":"Cohen","year":"2006","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522142141_DDR203C23","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1111\/j.1471-4159.2006.03895.x","article-title":"Expanded polyglutamines impair synaptic transmission and ubiquitin\u2014proteasome system in Caenorhabditis elegans","volume":"98","author":"Khan","year":"2006","journal-title":"J. Neurochem."},{"key":"key\n\t\t\t\t20170522142141_DDR203C24","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":"key\n\t\t\t\t20170522142141_DDR203C25","doi-asserted-by":"crossref","first-page":"10266","DOI":"10.1523\/JNEUROSCI.2734-04.2004","article-title":"A mutant ataxin-3 putative-cleavage fragment in brains of Machado\u2014Joseph disease patients and transgenic mice is cytotoxic above a critical concentration","volume":"24","author":"Goti","year":"2004","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522142141_DDR203C26","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":"key\n\t\t\t\t20170522142141_DDR203C27","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":"key\n\t\t\t\t20170522142141_DDR203C28","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\u2014Joseph disease lacking intranuclear inclusions and ataxin-3 cleavage products","volume":"40","author":"Silva-Fernandes","year":"2010","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522142141_DDR203C29","doi-asserted-by":"crossref","first-page":"2071","DOI":"10.1093\/hmg\/ddn106","article-title":"Striatal and nigral pathology in a lentiviral rat model of Machado\u2014Joseph disease","volume":"17","author":"Alves","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C30","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1098\/rstb.1976.0085","article-title":"The pharynx of Caenorhabditis elegans","volume":"275","author":"Albertson","year":"1976","journal-title":"Phil. Trans. R. Soc. Lond .B. Biol. Sci."},{"key":"key\n\t\t\t\t20170522142141_DDR203C31","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1098\/rstb.1976.0086","article-title":"The structure of the ventral nerve cord of Caenorhabditis elegans","volume":"275","author":"White","year":"1976","journal-title":"Phil. Trans. R. Soc. Lond. B. Biol. Sci."},{"key":"key\n\t\t\t\t20170522142141_DDR203C32","doi-asserted-by":"crossref","first-page":"7597","DOI":"10.1523\/JNEUROSCI.0990-06.2006","article-title":"Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model","volume":"26","author":"Brignull","year":"2006","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522142141_DDR203C33","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1073\/pnas.96.1.179","article-title":"Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron","volume":"96","author":"Faber","year":"1999","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522142141_DDR203C34","doi-asserted-by":"crossref","first-page":"5750","DOI":"10.1073\/pnas.100107297","article-title":"Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans","volume":"97","author":"Satyal","year":"2000","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522142141_DDR203C35","doi-asserted-by":"crossref","first-page":"13318","DOI":"10.1073\/pnas.231476398","article-title":"Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death","volume":"98","author":"Parker","year":"2001","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522142141_DDR203C36","doi-asserted-by":"crossref","first-page":"10417","DOI":"10.1073\/pnas.152161099","article-title":"The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans","volume":"99","author":"Morley","year":"2002","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522142141_DDR203C37","doi-asserted-by":"crossref","first-page":"1951","DOI":"10.1242\/dev.128.11.1951","article-title":"A regulatory cascade of three homeobox genes, ceh-10, ttx-3 and ceh-23, controls cell fate specification of a defined interneuron class in C. elegans","volume":"128","author":"Altun-Gultekin","year":"2001","journal-title":"Development"},{"key":"key\n\t\t\t\t20170522142141_DDR203C38","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1111\/j.1471-4159.2004.02369.x","article-title":"Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3","volume":"89","author":"Berke","year":"2004","journal-title":"J. Neurochem."},{"key":"key\n\t\t\t\t20170522142141_DDR203C39","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1016\/j.nbd.2007.06.005","article-title":"A mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado\u2014Joseph disease-like transgenic mice","volume":"27","author":"Colomer Gould","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522142141_DDR203C40","doi-asserted-by":"crossref","first-page":"18851","DOI":"10.1074\/jbc.M611914200","article-title":"Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3","volume":"282","author":"Haacke","year":"2007","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522142141_DDR203C41","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1093\/hmg\/ddi472","article-title":"Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3","volume":"15","author":"Haacke","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C42","first-page":"31","article-title":"An image processing application for quantification of protein aggregates in Caenorhabditis elegans","author":"Teixeira-Castro","year":"2011"},{"key":"key\n\t\t\t\t20170522142141_DDR203C43","doi-asserted-by":"crossref","first-page":"3006","DOI":"10.1101\/gad.1575307","article-title":"Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells","volume":"21","author":"Garcia","year":"2007","journal-title":"Genes Dev."},{"key":"key\n\t\t\t\t20170522142141_DDR203C44","doi-asserted-by":"crossref","first-page":"1471","DOI":"10.1126\/science.1124514","article-title":"Progressive disruption of cellular protein folding in models of polyglutamine diseases","volume":"311","author":"Gidalevitz","year":"2006","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522142141_DDR203C45","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1016\/0092-8674(93)80053-H","article-title":"Odorant-selective genes and neurons mediate olfaction in C. elegans","volume":"74","author":"Bargmann","year":"1993","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522142141_DDR203C46","doi-asserted-by":"crossref","first-page":"942","DOI":"10.1126\/science.277.5328.942","article-title":"daf-2, an insulin receptor-like gene that regulates longevity and diapause in Caenorhabditis elegans","volume":"277","author":"Kimura","year":"1997","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522142141_DDR203C47","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1126\/science.1089169","article-title":"Healthy animals with extreme longevity","volume":"302","author":"Arantes-Oliveira","year":"2003","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522142141_DDR203C48","doi-asserted-by":"crossref","first-page":"1319","DOI":"10.1126\/science.278.5341.1319","article-title":"daf-16: An HNF-3\/forkhead family member that can function to double the life-span of Caenorhabditis elegans","volume":"278","author":"Lin","year":"1997","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522142141_DDR203C49","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1038\/40194","article-title":"The Fork head transcription factor DAF-16 transduces insulin-like metabolic and longevity signals in C. elegans","volume":"389","author":"Ogg","year":"1997","journal-title":"Nature"},{"key":"key\n\t\t\t\t20170522142141_DDR203C50","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1111\/j.1474-9726.2008.00375.x","article-title":"Valproic acid extends Caenorhabditis elegans lifespan","volume":"7","author":"Evason","year":"2008","journal-title":"Aging Cell"},{"key":"key\n\t\t\t\t20170522142141_DDR203C51","doi-asserted-by":"crossref","first-page":"1937","DOI":"10.1534\/genetics.104.028423","article-title":"The L-type cyclin CYL-1 and the heat-shock-factor HSF-1 are required for heat-shock-induced protein expression in Caenorhabditis elegans","volume":"168","author":"Hajdu-Cronin","year":"2004","journal-title":"Genetics"},{"key":"key\n\t\t\t\t20170522142141_DDR203C52","doi-asserted-by":"crossref","first-page":"4843","DOI":"10.1093\/hmg\/ddp456","article-title":"Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3","volume":"18","author":"Jung","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C53","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1038\/ng0696-196","article-title":"Expanded polyglutamine in the Machado\u2014Joseph disease protein induces cell death in vitro and in vivo","volume":"13","author":"Ikeda","year":"1996","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C54","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1038\/sj.emboj.7601043","article-title":"An arginine\/lysine-rich motif is crucial for VCP\/p97-mediated modulation of ataxin-3 fibrillogenesis","volume":"25","author":"Boeddrich","year":"2006","journal-title":"EMBO J."},{"key":"key\n\t\t\t\t20170522142141_DDR203C55","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":"key\n\t\t\t\t20170522142141_DDR203C56","doi-asserted-by":"crossref","first-page":"2409","DOI":"10.1093\/hmg\/ddl164","article-title":"Ataxin-3 binds VCP\/p97 and regulates retrotranslocation of ERAD substrates","volume":"15","author":"Zhong","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C57","first-page":"272","article-title":"Neuronal intranuclear inclusions, dysregulation of cytokine expression and cell death in spinocerebellar ataxia type 3","volume":"25","author":"Evert","year":"2006","journal-title":"Clin. Neuropathol."},{"key":"key\n\t\t\t\t20170522142141_DDR203C58","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":"key\n\t\t\t\t20170522142141_DDR203C59","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":"key\n\t\t\t\t20170522142141_DDR203C60","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":"key\n\t\t\t\t20170522142141_DDR203C61","doi-asserted-by":"crossref","first-page":"e1000399","DOI":"10.1371\/journal.pgen.1000399","article-title":"Destabilizing protein polymorphisms in the genetic background direct phenotypic expression of mutant SOD1 toxicity","volume":"5","author":"Gidalevitz","year":"2009","journal-title":"PLoS Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C62","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1126\/science.1172871","article-title":"Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity","volume":"324","author":"Subramaniam","year":"2009","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522142141_DDR203C63","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1111\/j.1474-9726.2007.00289.x","article-title":"Changes in the structural complexity of the aged brain","volume":"6","author":"Dickstein","year":"2007","journal-title":"Aging Cell"},{"key":"key\n\t\t\t\t20170522142141_DDR203C64","doi-asserted-by":"crossref","first-page":"1157","DOI":"10.1016\/j.cell.2009.11.014","article-title":"Reduced IGF-1 signaling delays age-associated proteotoxicity in mice","volume":"139","author":"Cohen","year":"2009","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522142141_DDR203C65","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1126\/science.1143780","article-title":"Sirtuin 2 inhibitors rescue alpha-synuclein-mediated toxicity in models of Parkinson's disease","volume":"317","author":"Outeiro","year":"2007","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522142141_DDR203C66","doi-asserted-by":"crossref","first-page":"e1000027","DOI":"10.1371\/journal.pgen.1000027","article-title":"C. elegans model identifies genetic modifiers of alpha-synuclein inclusion formation during aging","volume":"4","author":"van Ham","year":"2008","journal-title":"PLoS Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C67","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1038\/ng1534","article-title":"Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons","volume":"37","author":"Parker","year":"2005","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522142141_DDR203C68","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1111\/j.1474-9726.2009.00541.x","article-title":"Temporal requirements of insulin\/IGF-1 signaling for proteotoxicity protection","volume":"9","author":"Cohen","year":"2010","journal-title":"Aging Cell"},{"key":"key\n\t\t\t\t20170522142141_DDR203C69","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":"key\n\t\t\t\t20170522142141_DDR203C70","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1016\/j.nbd.2008.10.016","article-title":"In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis","volume":"33","author":"Williams","year":"2009","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522142141_DDR203C71","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1093\/genetics\/77.1.71","article-title":"The genetics of Caenorhabditis elegans","volume":"77","author":"Brenner","year":"1974","journal-title":"Genetics"},{"key":"key\n\t\t\t\t20170522142141_DDR203C72","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/S0076-6879(06)12016-9","article-title":"Modeling polyglutamine pathogenesis in C. elegans","volume":"412","author":"Brignull","year":"2006","journal-title":"Methods Enzymol."},{"key":"key\n\t\t\t\t20170522142141_DDR203C73","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/S0091-679X(08)61381-3","article-title":"Basic culture methods","volume":"48","author":"Lewis","year":"1995","journal-title":"Methods Cell Biol."},{"key":"key\n\t\t\t\t20170522142141_DDR203C74","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1038\/35007077","article-title":"High mobility of proteins in the mammalian cell nucleus","volume":"404","author":"Phair","year":"2000","journal-title":"Nature"},{"key":"key\n\t\t\t\t20170522142141_DDR203C75","doi-asserted-by":"crossref","first-page":"e504","DOI":"10.1371\/journal.pone.0000504","article-title":"Identification of potential therapeutic drugs for Huntington's disease using Caenorhabditis elegans","volume":"2","author":"Voisine","year":"2007","journal-title":"PLoS One"}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/hmg\/article\/20\/15\/2996\/2527067\/Neuronspecific-proteotoxicity-of-mutant-ataxin3-in","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,22]],"date-time":"2021-11-22T20:24:33Z","timestamp":1637612673000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddr203"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,5,5]]},"references-count":75,"journal-issue":{"issue":"15","published-online":{"date-parts":[[2011,5,5]]},"published-print":{"date-parts":[[2011,8,1]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddr203","relation":{},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published":{"date-parts":[[2011,5,5]]}}}