{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T13:57:24Z","timestamp":1790171844081,"version":"4.1.0"},"reference-count":39,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["1R01 AG034228"],"award-info":[{"award-number":["1R01 AG034228"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["R01 NS038712"],"award-info":[{"award-number":["R01 NS038712"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"UM Protein Folding Diseases Initiative"}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2015,10]]},"DOI":"10.1016\/j.nbd.2015.06.017","type":"journal-article","created":{"date-parts":[[2015,7,3]],"date-time":"2015-07-03T03:47:33Z","timestamp":1435895253000},"page":"281-288","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":26,"special_numbering":"C","title":["Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3"],"prefix":"10.1016","volume":"82","author":[{"given":"Li","family":"Zeng","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean A.","family":"Merillat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eiko N.","family":"Minakawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew D.","family":"Perkins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biswarathan","family":"Ramani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara J.","family":"Tallaksen-Greene","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria do Carmo","family":"Costa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger L.","family":"Albin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henry L.","family":"Paulson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2015.06.017_bb0005","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1007\/s00401-012-0970-z","article-title":"Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion","volume":"123","author":"Brettschneider","year":"2012","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.nbd.2015.06.017_bb0010","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.2015.06.017_bb0015","doi-asserted-by":"crossref","first-page":"9310","DOI":"10.1073\/pnas.152101299","article-title":"Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis","volume":"99","author":"Chai","year":"2002","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2015.06.017_bb0020","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1038\/mt.2013.144","article-title":"Toward RNAi therapy for the polyglutamine disease Machado\u2013Joseph disease","volume":"21","author":"Costa","year":"2013","journal-title":"Mol. Ther."},{"key":"10.1016\/j.nbd.2015.06.017_bb0025","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/nature10353","article-title":"Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS\/dementia","volume":"477","author":"Deng","year":"2011","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2015.06.017_bb0030","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/j.febslet.2004.06.077","article-title":"Identification of ubiquitin-interacting proteins in purified polyglutamine aggregates","volume":"571","author":"Doi","year":"2004","journal-title":"FEBS Lett."},{"key":"10.1016\/j.nbd.2015.06.017_bb0035","doi-asserted-by":"crossref","first-page":"2565","DOI":"10.1093\/hmg\/ddv020","article-title":"ALS-linked mutations in ubiquilin-2 or hnRNPA1 reduce interaction between ubiquilin-2 and hnRNPA1","volume":"24","author":"Gilpin","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2015.06.017_bb0040","doi-asserted-by":"crossref","first-page":"1252","DOI":"10.1038\/sj.embor.7400823","article-title":"The UBL domain of PLIC-1 regulates aggresome formation","volume":"7","author":"Heir","year":"2006","journal-title":"EMBO Rep."},{"key":"10.1016\/j.nbd.2015.06.017_bb0045","doi-asserted-by":"crossref","first-page":"3702","DOI":"10.1093\/hmg\/ddq285","article-title":"Early autophagic response in a novel knock-in model of Huntington disease","volume":"19","author":"Heng","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2015.06.017_bb0050","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1016\/S1097-2765(00)00040-X","article-title":"The hPLIC proteins may provide a link between the ubiquitination machinery and the proteasome","volume":"6","author":"Kleijnen","year":"2000","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2015.06.017_bb0055","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/j.febslet.2004.04.031","article-title":"Ubiquilin interacts with ubiquitylated proteins and proteasome through its ubiquitin-associated and ubiquitin-like domains","volume":"566","author":"Ko","year":"2004","journal-title":"FEBS Lett."},{"key":"10.1016\/j.nbd.2015.06.017_bb0060","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1515\/hsz-2012-0120","article-title":"Ubiquilins in the crosstalk among proteolytic pathways","volume":"393","author":"Lee","year":"2012","journal-title":"Biol. Chem."},{"key":"10.1016\/j.nbd.2015.06.017_bb0065","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1083\/jcb.200903024","article-title":"Ubiquilin and p97\/VCP bind erasin, forming a complex involved in ERAD","volume":"187","author":"Lim","year":"2009","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2015.06.017_bb0070","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1093\/hmg\/10.2.137","article-title":"Neurological abnormalities in a knock-in mouse model of Huntington's disease","volume":"10","author":"Lin","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2015.06.017_bb0075","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1083\/jcb.151.4.847","article-title":"Identification of ubiquilin, a novel presenilin interactor that increases presenilin protein accumulation","volume":"151","author":"Mah","year":"2000","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2015.06.017_bb0080","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1007\/s00401-012-0999-z","article-title":"Ubiquilin immunoreactivity in cytoplasmic and nuclear inclusions in synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease","volume":"124","author":"Mori","year":"2012","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.nbd.2015.06.017_bb0085","doi-asserted-by":"crossref","first-page":"2459","DOI":"10.2174\/092986711795843236","article-title":"Removing protein aggregates: the role of proteolysis in neurodegeneration","volume":"18","author":"Nijholt","year":"2011","journal-title":"Curr. Med. Chem."},{"key":"10.1016\/j.nbd.2015.06.017_bb0200","doi-asserted-by":"crossref","first-page":"e76598","DOI":"10.1371\/journal.pone.0076598","article-title":"Ubiquilin 2 is not associated with tau pathology","volume":"8","author":"Nolle","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2015.06.017_bb0095","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0092-8674(00)80617-0","article-title":"Reversing neurodegeneration: a promise unfolds","volume":"101","author":"Orr","year":"2000","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2015.06.017_bb0205","first-page":"1617","article-title":"Evidence of a link between ubiquilin 2 and optineurin in amyotrophic lateral sclerosis","author":"Osaka","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2015.06.017_bb0105","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.2015.06.017_bb0210","first-page":"1211","article-title":"A knock-in mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript","author":"Ramani","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2015.06.017_bb0115","doi-asserted-by":"crossref","first-page":"3219","DOI":"10.1093\/hmg\/ddq231","article-title":"Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy","volume":"19","author":"Rothenberg","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2015.06.017_bb0120","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1038\/nature05291","article-title":"The roles of intracellular protein-degradation pathways in neurodegeneration","volume":"443","author":"Rubinsztein","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2015.06.017_bb0125","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1016\/j.brainres.2013.06.006","article-title":"Unbiased screen reveals ubiquilin-1 and -2 highly associated with huntingtin inclusions","volume":"1524","author":"Rutherford","year":"2013","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2015.06.017_bb0215","doi-asserted-by":"crossref","first-page":"e87513","DOI":"10.1371\/journal.pone.0087513","article-title":"Ubiquilin-1 overexpression increases the lifespan and delays accumulation of Huntingtin aggregates in the R6\/2 mouse model of Huntington's disease","volume":"9","author":"Safren","year":"2014","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2015.06.017_bb0130","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.brainres.2014.12.008","article-title":"Signature changes in ubiquilin expression in the R6\/2 mouse model of Huntington's disease","volume":"1597","author":"Safren","year":"2015","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2015.06.017_bb0140","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1111\/nan.12036","article-title":"Ubiquilin-1 immunoreactivity is concentrated on Hirano bodies and dystrophic neurites in Alzheimer's disease brains","volume":"39","author":"Satoh","year":"2013","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.nbd.2015.06.017_bb0145","doi-asserted-by":"crossref","first-page":"35689","DOI":"10.1074\/jbc.M111.243147","article-title":"Ubiquilin-1 is a molecular chaperone for the amyloid precursor protein","volume":"286","author":"Stieren","year":"2011","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2015.06.017_bb0150","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1111\/j.1600-0854.2010.01149.x","article-title":"Alzheimer's disease-associated ubiquilin-1 regulates presenilin-1 accumulation and aggresome formation","volume":"12","author":"Viswanathan","year":"2011","journal-title":"Traffic"},{"key":"10.1016\/j.nbd.2015.06.017_bb0155","doi-asserted-by":"crossref","first-page":"1767","DOI":"10.1021\/bi011892y","article-title":"Structural studies of the interaction between ubiquitin family proteins and proteasome subunit S5a","volume":"41","author":"Walters","year":"2002","journal-title":"Biochemistry"},{"key":"10.1016\/j.nbd.2015.06.017_bb0165","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1016\/j.bbrc.2007.06.097","article-title":"Ubiquilin interacts and enhances the degradation of expanded-polyglutamine proteins","volume":"360","author":"Wang","year":"2007","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.nbd.2015.06.017_bb0160","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1093\/hmg\/ddl017","article-title":"Suppression of polyglutamine-induced toxicity in cell and animal models of Huntington's disease by ubiquilin","volume":"15","author":"Wang","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2015.06.017_bb0170","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/S1097-2765(00)80212-9","article-title":"Ubiquitin-related proteins regulate interaction of vimentin intermediate filaments with the plasma membrane","volume":"4","author":"Wu","year":"1999","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2015.06.017_bb0175","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1016\/j.yexcr.2006.10.031","article-title":"Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin","volume":"313","author":"Yang","year":"2007","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.nbd.2015.06.017_bb0185","doi-asserted-by":"crossref","first-page":"2467","DOI":"10.1111\/j.1742-4658.2012.08627.x","article-title":"Laforin is required for the functional activation of malin in endoplasmic reticulum stress resistance in neuronal cells","volume":"279","author":"Zeng","year":"2012","journal-title":"FEBS J."},{"key":"10.1016\/j.nbd.2015.06.017_bb0180","doi-asserted-by":"crossref","first-page":"201","DOI":"10.3233\/JHD-130058","article-title":"The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease","volume":"2","author":"Zeng","year":"2013","journal-title":"J. Huntingt. Dis."},{"key":"10.1016\/j.nbd.2015.06.017_bb0190","first-page":"18","article-title":"An emerging role for ubiquilin 1 in regulating protein quality control system and in disease pathogenesis","volume":"8","author":"Zhang","year":"2009","journal-title":"Discov. Med."},{"key":"10.1016\/j.nbd.2015.06.017_bb0195","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.biocel.2014.02.018","article-title":"Ubiquilin 2: a component of the ubiquitin\u2013proteasome system with an emerging role in neurodegeneration","volume":"50","author":"Zhang","year":"2014","journal-title":"Int. J. Biochem. Cell Biol."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996115300012?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996115300012?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T13:28:00Z","timestamp":1599226080000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996115300012"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,10]]},"references-count":39,"alternative-id":["S0969996115300012"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2015.06.017","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2015,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2015.06.017","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2015 Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}