{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T21:03:52Z","timestamp":1783458232773,"version":"3.55.0"},"reference-count":126,"publisher":"Cold Spring Harbor Laboratory","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cold Spring Harb Perspect Med"],"published-print":{"date-parts":[[2018,2]]},"DOI":"10.1101\/cshperspect.a024257","type":"journal-article","created":{"date-parts":[[2017,1,18]],"date-time":"2017-01-18T02:31:16Z","timestamp":1484706676000},"page":"a024257","source":"Crossref","is-referenced-by-count":54,"title":["Prion-Like Characteristics of Polyglutamine-Containing Proteins"],"prefix":"10.1101","volume":"8","author":[{"given":"Margaret M.P.","family":"Pearce","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ron R.","family":"Kopito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"246","published-online":{"date-parts":[[2017,1,17]]},"reference":[{"key":"2018091410374253000_8.2.a024257.1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-014-1254-6"},{"key":"2018091410374253000_8.2.a024257.2","doi-asserted-by":"publisher","DOI":"10.1038\/214764a0"},{"key":"2018091410374253000_8.2.a024257.3","doi-asserted-by":"publisher","DOI":"10.1039\/C4CP05563B"},{"key":"2018091410374253000_8.2.a024257.4","doi-asserted-by":"publisher","DOI":"10.1038\/nature02998"},{"key":"2018091410374253000_8.2.a024257.5","doi-asserted-by":"publisher","DOI":"10.1038\/nchembio.1563"},{"key":"2018091410374253000_8.2.a024257.6","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.182393899"},{"key":"2018091410374253000_8.2.a024257.7","doi-asserted-by":"publisher","DOI":"10.1038\/nature06022"},{"key":"2018091410374253000_8.2.a024257.8","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2005.10.053"},{"key":"2018091410374253000_8.2.a024257.9","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.0030177"},{"key":"2018091410374253000_8.2.a024257.10","doi-asserted-by":"publisher","DOI":"10.1126\/science.1123511"},{"key":"2018091410374253000_8.2.a024257.11","doi-asserted-by":"publisher","DOI":"10.1126\/science.6815801"},{"key":"2018091410374253000_8.2.a024257.12","doi-asserted-by":"publisher","DOI":"10.1007\/BF00308809"},{"key":"2018091410374253000_8.2.a024257.13","doi-asserted-by":"publisher","DOI":"10.1007\/s00441-004-0956-9"},{"key":"2018091410374253000_8.2.a024257.14","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2013.05.001"},{"key":"2018091410374253000_8.2.a024257.15","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23937"},{"key":"2018091410374253000_8.2.a024257.16","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2164-8-126"},{"key":"2018091410374253000_8.2.a024257.17","doi-asserted-by":"publisher","DOI":"10.1006\/jmbi.2001.4850"},{"key":"2018091410374253000_8.2.a024257.18","doi-asserted-by":"publisher","DOI":"10.1021\/bi011772q"},{"key":"2018091410374253000_8.2.a024257.19","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.182276099"},{"key":"2018091410374253000_8.2.a024257.20","doi-asserted-by":"publisher","DOI":"10.1038\/nchembio.131"},{"key":"2018091410374253000_8.2.a024257.21","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24174"},{"key":"2018091410374253000_8.2.a024257.22","doi-asserted-by":"publisher","DOI":"10.1038\/ncb1901"},{"key":"2018091410374253000_8.2.a024257.23","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1301175110"},{"key":"2018091410374253000_8.2.a024257.24","doi-asserted-by":"publisher","DOI":"10.1016\/0022-2836(77)90214-5"},{"key":"2018091410374253000_8.2.a024257.25","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.126086"},{"key":"2018091410374253000_8.2.a024257.26","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.94.18.9773"},{"key":"2018091410374253000_8.2.a024257.27","doi-asserted-by":"publisher","DOI":"10.1016\/j.sbi.2015.03.008"},{"key":"2018091410374253000_8.2.a024257.28","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2011.11.033"},{"key":"2018091410374253000_8.2.a024257.29","doi-asserted-by":"publisher","DOI":"10.1016\/j.jchemneu.2004.02.005"},{"key":"2018091410374253000_8.2.a024257.30","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0903691106"},{"key":"2018091410374253000_8.2.a024257.31","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0007275"},{"key":"2018091410374253000_8.2.a024257.32","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-387"},{"key":"2018091410374253000_8.2.a024257.33","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2012.02.022"},{"key":"2018091410374253000_8.2.a024257.34","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh278"},{"key":"2018091410374253000_8.2.a024257.35","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/cdf573"},{"key":"2018091410374253000_8.2.a024257.36","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.318949"},{"key":"2018091410374253000_8.2.a024257.37","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-012-8315-4"},{"key":"2018091410374253000_8.2.a024257.38","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2010.11.042"},{"key":"2018091410374253000_8.2.a024257.39","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M808759200"},{"key":"2018091410374253000_8.2.a024257.40","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.4000-11.2011"},{"key":"2018091410374253000_8.2.a024257.41","doi-asserted-by":"crossref","first-page":"4897","DOI":"10.1523\/JNEUROSCI.22-12-04897.2002","article-title":"Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice","volume":"22","year":"2002","journal-title":"J Neurosci"},{"key":"2018091410374253000_8.2.a024257.42","doi-asserted-by":"publisher","DOI":"10.1088\/0953-8984\/25\/37\/373101"},{"key":"2018091410374253000_8.2.a024257.43","doi-asserted-by":"publisher","DOI":"10.1038\/ng1542"},{"key":"2018091410374253000_8.2.a024257.44","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0009642"},{"key":"2018091410374253000_8.2.a024257.45","doi-asserted-by":"publisher","DOI":"10.1038\/nature10875"},{"key":"2018091410374253000_8.2.a024257.46","doi-asserted-by":"publisher","DOI":"10.1172\/JCI43366"},{"key":"2018091410374253000_8.2.a024257.47","doi-asserted-by":"publisher","DOI":"10.1371\/currents.RRN1210"},{"key":"2018091410374253000_8.2.a024257.48","doi-asserted-by":"publisher","DOI":"10.1016\/j.tcb.2014.05.003"},{"key":"2018091410374253000_8.2.a024257.49","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1301440110"},{"key":"2018091410374253000_8.2.a024257.50","doi-asserted-by":"publisher","DOI":"10.3109\/10409238.2014.914151"},{"key":"2018091410374253000_8.2.a024257.51","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E"},{"key":"2018091410374253000_8.2.a024257.52","doi-asserted-by":"publisher","DOI":"10.1038\/79162"},{"key":"2018091410374253000_8.2.a024257.53","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2642-12.2013"},{"key":"2018091410374253000_8.2.a024257.54","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a024240"},{"key":"2018091410374253000_8.2.a024257.55","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1412504111"},{"key":"2018091410374253000_8.2.a024257.56","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-biochem-060208-092442"},{"key":"2018091410374253000_8.2.a024257.57","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81781-X"},{"key":"2018091410374253000_8.2.a024257.58","doi-asserted-by":"publisher","DOI":"10.1097\/NEN.0b013e31818c3618"},{"key":"2018091410374253000_8.2.a024257.59","doi-asserted-by":"publisher","DOI":"10.1038\/nm1747"},{"key":"2018091410374253000_8.2.a024257.60","doi-asserted-by":"publisher","DOI":"10.1038\/nature03679"},{"key":"2018091410374253000_8.2.a024257.61","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.97.4.1589"},{"key":"2018091410374253000_8.2.a024257.62","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2013.05.003"},{"key":"2018091410374253000_8.2.a024257.63","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.b.30992"},{"key":"2018091410374253000_8.2.a024257.64","doi-asserted-by":"publisher","DOI":"10.1038\/srep00700"},{"key":"2018091410374253000_8.2.a024257.65","doi-asserted-by":"publisher","DOI":"10.1038\/nm1746"},{"key":"2018091410374253000_8.2.a024257.66","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2011.12.032"},{"key":"2018091410374253000_8.2.a024257.67","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0031302"},{"key":"2018091410374253000_8.2.a024257.68","doi-asserted-by":"publisher","DOI":"10.1126\/science.1227157"},{"key":"2018091410374253000_8.2.a024257.69","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2012.01.004"},{"key":"2018091410374253000_8.2.a024257.70","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0406847102"},{"key":"2018091410374253000_8.2.a024257.71","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81369-0"},{"key":"2018091410374253000_8.2.a024257.72","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.1.13"},{"key":"2018091410374253000_8.2.a024257.73","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(83)90207-6"},{"key":"2018091410374253000_8.2.a024257.74","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200112104"},{"key":"2018091410374253000_8.2.a024257.75","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.152161099"},{"key":"2018091410374253000_8.2.a024257.76","doi-asserted-by":"crossref","first-page":"4050","DOI":"10.1523\/JNEUROSCI.20-11-04050.2000","article-title":"High-level neuronal expression of A\u03b21\u201342 in wild-type human amyloid protein precursor transgenic mice: Synaptotoxicity without plaque formation","volume":"20","year":"2000","journal-title":"J Neurosci"},{"key":"2018091410374253000_8.2.a024257.77","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.1000034"},{"key":"2018091410374253000_8.2.a024257.78","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1017275108"},{"key":"2018091410374253000_8.2.a024257.79","doi-asserted-by":"publisher","DOI":"10.1038\/nature03680"},{"key":"2018091410374253000_8.2.a024257.80","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2013.06.007"},{"key":"2018091410374253000_8.2.a024257.81","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2013.06.034"},{"key":"2018091410374253000_8.2.a024257.82","doi-asserted-by":"publisher","DOI":"10.1126\/science.273.5275.622"},{"key":"2018091410374253000_8.2.a024257.83","doi-asserted-by":"publisher","DOI":"10.1136\/vr.80.1.2"},{"key":"2018091410374253000_8.2.a024257.84","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms7768"},{"key":"2018091410374253000_8.2.a024257.85","doi-asserted-by":"publisher","DOI":"10.1038\/nn.3761"},{"key":"2018091410374253000_8.2.a024257.86","doi-asserted-by":"publisher","DOI":"10.1016\/j.bpj.2012.04.050"},{"key":"2018091410374253000_8.2.a024257.87","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.biochem.052308.114844"},{"key":"2018091410374253000_8.2.a024257.88","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1106557108"},{"key":"2018091410374253000_8.2.a024257.89","doi-asserted-by":"publisher","DOI":"10.1098\/rstb.1999.0455"},{"key":"2018091410374253000_8.2.a024257.90","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-genet-110711-155524"},{"key":"2018091410374253000_8.2.a024257.91","doi-asserted-by":"publisher","DOI":"10.1038\/ncb1830"},{"key":"2018091410374253000_8.2.a024257.92","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr251"},{"key":"2018091410374253000_8.2.a024257.93","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1421313111"},{"key":"2018091410374253000_8.2.a024257.94","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn025"},{"key":"2018091410374253000_8.2.a024257.95","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.24.16.7206-7213.2004"},{"key":"2018091410374253000_8.2.a024257.96","first-page":"137","article-title":"Binding, internalization and fate of Huntingtin Exon1 fibrillar assemblies in mitotic and non-mitotic neuroblastoma cells","volume":"42","year":"2015","journal-title":"Neuropathol Appl Neurobiol"},{"key":"2018091410374253000_8.2.a024257.97","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gks011"},{"key":"2018091410374253000_8.2.a024257.98","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80514-0"},{"key":"2018091410374253000_8.2.a024257.99","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0023841"},{"key":"2018091410374253000_8.2.a024257.100","doi-asserted-by":"publisher","DOI":"10.1038\/nrg1616"},{"key":"2018091410374253000_8.2.a024257.101","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2010.01.008"},{"key":"2018091410374253000_8.2.a024257.102","doi-asserted-by":"publisher","DOI":"10.1016\/j.semcdb.2015.03.003"},{"key":"2018091410374253000_8.2.a024257.103","doi-asserted-by":"publisher","DOI":"10.1126\/science.1092194"},{"key":"2018091410374253000_8.2.a024257.104","doi-asserted-by":"crossref","first-page":"5703","DOI":"10.1523\/JNEUROSCI.21-15-05703.2001","article-title":"Generation of aggregated \u03b2-amyloid in the rat hippocampus impairs synaptic transmission and plasticity and causes memory deficits","volume":"21","year":"2001","journal-title":"J Neurosci"},{"key":"2018091410374253000_8.2.a024257.105","doi-asserted-by":"publisher","DOI":"10.1126\/science.1172871"},{"key":"2018091410374253000_8.2.a024257.106","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1093\/genetics\/137.3.671","article-title":"The SUP35 omnipotent suppressor gene is involved in the maintenance of the non-Mendelian determinant [psi +] in the yeast Saccharomyces cerevisiae","volume":"137","year":"1994","journal-title":"Genetics"},{"key":"2018091410374253000_8.2.a024257.107","doi-asserted-by":"publisher","DOI":"10.1038\/nsmb.1570"},{"key":"2018091410374253000_8.2.a024257.108","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.19.13666"},{"key":"2018091410374253000_8.2.a024257.109","doi-asserted-by":"publisher","DOI":"10.1038\/nature06108"},{"key":"2018091410374253000_8.2.a024257.110","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112.372474"},{"key":"2018091410374253000_8.2.a024257.111","doi-asserted-by":"publisher","DOI":"10.1038\/35035005"},{"key":"2018091410374253000_8.2.a024257.112","doi-asserted-by":"publisher","DOI":"10.1110\/ps.062609607"},{"key":"2018091410374253000_8.2.a024257.113","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddv174"},{"key":"2018091410374253000_8.2.a024257.114","doi-asserted-by":"publisher","DOI":"10.1038\/nchembio.1308"},{"key":"2018091410374253000_8.2.a024257.115","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2011.08.033"},{"key":"2018091410374253000_8.2.a024257.116","doi-asserted-by":"publisher","DOI":"10.1038\/416535a"},{"key":"2018091410374253000_8.2.a024257.117","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81200-3"},{"key":"2018091410374253000_8.2.a024257.118","doi-asserted-by":"publisher","DOI":"10.1126\/science.7909170"},{"key":"2018091410374253000_8.2.a024257.119","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1100976108"},{"key":"2018091410374253000_8.2.a024257.120","doi-asserted-by":"publisher","DOI":"10.1038\/nrm3920"},{"key":"2018091410374253000_8.2.a024257.121","doi-asserted-by":"crossref","first-page":"e93891","DOI":"10.1371\/journal.pone.0093891","article-title":"Large-scale RNA interference screening in mammalian cells identifies novel regulators of mutant huntingtin aggregation","volume":"9","year":"2014","journal-title":"PLoS ONE"},{"key":"2018091410374253000_8.2.a024257.122","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.23.2905"},{"key":"2018091410374253000_8.2.a024257.123","doi-asserted-by":"publisher","DOI":"10.1016\/S0361-9230(01)00578-0"},{"key":"2018091410374253000_8.2.a024257.124","doi-asserted-by":"crossref","first-page":"2193","DOI":"10.1523\/JNEUROSCI.23-06-02193.2003","article-title":"Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington\u2019s disease","volume":"23","year":"2003","journal-title":"J Neurosci"},{"key":"2018091410374253000_8.2.a024257.125","doi-asserted-by":"publisher","DOI":"10.1016\/j.tips.2013.12.001"},{"key":"2018091410374253000_8.2.a024257.126","doi-asserted-by":"publisher","DOI":"10.1534\/genetics.109.112516"}],"container-title":["Cold Spring Harbor Perspectives in Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1101\/cshperspect.a024257","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,7,21]],"date-time":"2022-07-21T23:29:28Z","timestamp":1658446168000},"score":1,"resource":{"primary":{"URL":"http:\/\/perspectivesinmedicine.cshlp.org\/lookup\/doi\/10.1101\/cshperspect.a024257"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,1,17]]},"references-count":126,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2018,2,1]]},"published-print":{"date-parts":[[2018,2]]}},"alternative-id":["10.1101\/cshperspect.a024257"],"URL":"https:\/\/doi.org\/10.1101\/cshperspect.a024257","relation":{},"ISSN":["2157-1422"],"issn-type":[{"value":"2157-1422","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,1,17]]}}}