{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,13]],"date-time":"2026-04-13T15:02:18Z","timestamp":1776092538534,"version":"3.50.1"},"reference-count":49,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[1998,10,1]],"date-time":"1998-10-01T00:00:00Z","timestamp":907200000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[1998,10,1]],"date-time":"1998-10-01T00:00:00Z","timestamp":907200000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2013,7,17]],"date-time":"2013-07-17T00:00:00Z","timestamp":1374019200000},"content-version":"vor","delay-in-days":5403,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Cell"],"published-print":{"date-parts":[[1998,10]]},"DOI":"10.1016\/s0092-8674(00)81781-x","type":"journal-article","created":{"date-parts":[[2004,6,23]],"date-time":"2004-06-23T10:58:07Z","timestamp":1087988287000},"page":"41-53","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":787,"title":["Ataxin-1 Nuclear Localization and Aggregation"],"prefix":"10.1016","volume":"95","author":[{"given":"Ivan A","family":"Klement","sequence":"first","affiliation":[]},{"given":"Pamela J","family":"Skinner","sequence":"additional","affiliation":[]},{"given":"Michael D","family":"Kaytor","sequence":"additional","affiliation":[]},{"given":"Hong","family":"Yi","sequence":"additional","affiliation":[]},{"given":"Steven M","family":"Hersch","sequence":"additional","affiliation":[]},{"given":"H.Brent","family":"Clark","sequence":"additional","affiliation":[]},{"given":"Huda Y","family":"Zoghbi","sequence":"additional","affiliation":[]},{"given":"Harry T","family":"Orr","sequence":"additional","affiliation":[]}],"member":"78","reference":[{"key":"10.1016\/S0092-8674(00)81781-X_BIB1","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1002\/mds.870120408","article-title":"Dentatorubral and pallidoluysian atrophy (DRPLA). Clinical and neuropathological findings in genetically confirmed North American and European pedigrees","volume":"12","author":"Becher","year":"1997","journal-title":"Mov. Disord."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB2","doi-asserted-by":"crossref","first-page":"1873","DOI":"10.1242\/jcs.108.5.1873","article-title":"Colocalization of a high molecular mass phoshpoprotein of the nuclear matrix (p255) with spliceosomes","volume":"108","author":"Bisotto","year":"1995","journal-title":"J. Cell Sci."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB3","first-page":"193","article-title":"Nuclear localization signals","volume":"3","author":"Boulikas","year":"1993","journal-title":"Crit. Rev. Eukaryot. Gene Expr."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB4","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1016\/0092-8674(95)90273-2","article-title":"SCA1 transgenic mice","volume":"82","author":"Burright","year":"1995","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB5","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1093\/hmg\/6.4.513","article-title":"Identification of a self-association region within the SCA1 gene product, ataxin-1","volume":"6","author":"Burright","year":"1997","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB6","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1016\/0003-2697(87)90021-2","article-title":"Single-step of RNA isolation by acid quanidinium thiocyanate-phenol-chloroform extraction","volume":"162","author":"Chomczynski","year":"1987","journal-title":"Anal. Biochem."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB7","doi-asserted-by":"crossref","first-page":"7385","DOI":"10.1523\/JNEUROSCI.17-19-07385.1997","article-title":"Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations","volume":"17","author":"Clark","year":"1997","journal-title":"J. Neurosci."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB8","doi-asserted-by":"crossref","first-page":"1519","DOI":"10.1093\/hmg\/6.9.1519","article-title":"Huntingtin-associated protein 1 (HAP1) binds to a Trio-like polypeptide, with a rac1 guanine nucleotide exchange factor domain","volume":"6","author":"Colomer","year":"1997","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB9","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1038\/502","article-title":"Chaperone suppression of ataxin-1 aggregation and altered subcellular proteosome localization imply protein misfolding in SCA1","volume":"19","author":"Cummings","year":"1998","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB10","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1038\/ng0997-65","article-title":"Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion","volume":"17","author":"David","year":"1997","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB11","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/S0092-8674(00)80513-9","article-title":"Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation","volume":"90","author":"Davies","year":"1997","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB12","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/S0140-6736(97)08360-8","article-title":"Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?","volume":"351","author":"Davies","year":"1998","journal-title":"Lancet"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB13","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1016\/0896-6273(95)90346-1","article-title":"Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons","volume":"14","author":"DiFiglia","year":"1995","journal-title":"Neuron"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB14","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain","volume":"277","author":"DiFiglia","year":"1997","journal-title":"Science"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB15","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1242\/jcs.105.2.481","article-title":"Use of a general purpose mammalian expression vector for studying intracellular protein targeting","volume":"105","author":"Frangioni","year":"1993","journal-title":"J. Cell Sci."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB16","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/0092-8674(95)90420-4","article-title":"Nuclear export signals and the fast track to the cytoplasm","volume":"82","author":"Gerace","year":"1995","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB17","doi-asserted-by":"crossref","first-page":"8710","DOI":"10.1073\/pnas.92.19.8710","article-title":"Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies","volume":"92","author":"Gutekunst","year":"1995","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB18","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes","volume":"72","author":"Huntington\u2019s Disease Collaborative Research Group","year":"1993","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB19","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1038\/ng0696-196","article-title":"Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo","volume":"13","author":"Ikeda","year":"1996","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB20","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1038\/ng1196-285","article-title":"Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG\/glutamine repeats","volume":"14","author":"Imbert","year":"1996","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB21","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1038\/311033a0","article-title":"Sequence requirements for nuclear location of simian virus 40 large-T antigen","volume":"311","author":"Kalderon","year":"1984","journal-title":"Nature"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB22","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/0092-8674(84)90457-4","article-title":"A short amino acid sequence able to specify nuclear localization","volume":"39","author":"Kalderon","year":"1984","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB23","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\/S0092-8674(00)81781-X_BIB24","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1038\/ng0194-9","article-title":"Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)","volume":"6","author":"Koide","year":"1994","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB25","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1038\/ng0198-72","article-title":"Rapid cloning of expanded trinucleotide repeat sequences from genomic DNA","volume":"18","author":"Koob","year":"1998","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB26","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1093\/hmg\/5.9.1311","article-title":"Spinocerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase","volume":"5","author":"Koshy","year":"1996","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB27","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/352077a0","article-title":"Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy","volume":"352","author":"La Spada","year":"1991","journal-title":"Nature"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB28","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/S0092-8674(00)81369-0","article-title":"Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice","volume":"87","author":"Mangiarini","year":"1996","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB29","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1038\/40159","article-title":"The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1","volume":"389","author":"Matilla","year":"1997","journal-title":"Nature"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB30","article-title":"Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation","volume":"in press","author":"Matilla","year":"1998","journal-title":"J. Neurosci."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB31","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1038\/ng0194-14","article-title":"Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p","volume":"6","author":"Nagafuchi","year":"1994","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB32","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1016\/S0092-8674(00)80464-X","article-title":"Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse","volume":"91","author":"Ordway","year":"1997","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB33","article-title":"Toward understanding polyglutamine-induced neurological disease in spinocerebellar ataxia type 1","volume":"649\u2013657","author":"Orr","year":"1996","journal-title":"Cold Spring Harbor Symp. Quant. Biol. LXI"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB34","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/ng0793-221","article-title":"Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1","volume":"4","author":"Orr","year":"1993","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB35","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\/S0092-8674(00)81781-X_BIB36","doi-asserted-by":"crossref","first-page":"5355","DOI":"10.1073\/pnas.91.12.5355","article-title":"Glutamine repeats as polar zippers","volume":"91","author":"Perutz","year":"1994","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB37","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1038\/ng1196-269","article-title":"Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2","volume":"14","author":"Pulst","year":"1996","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB38","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/0092-8674(84)90035-7","article-title":"The intranuclear location of a herpes simplex virus DNA-binding protein is determined by the status of viral DNA replication","volume":"36","author":"Quinlan","year":"1984","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB39","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1016\/S0896-6273(00)80405-5","article-title":"Intranuclear neuronal inclusions","volume":"19","author":"Ross","year":"1997","journal-title":"Neuron"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB40","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1038\/ng1196-277","article-title":"Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT","volume":"14","author":"Sanpei","year":"1996","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB41","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/S0092-8674(00)81782-1","article-title":"Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions","volume":"95","author":"Saudou","year":"1998","journal-title":"Cell"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB42","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1038\/ng0595-94","article-title":"Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals","volume":"10","author":"Servadio","year":"1995","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB43","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1016\/0896-6273(95)90345-3","article-title":"Widespread expression of Huntington\u2019s disease gene (IT15) protein product","volume":"14","author":"Sharp","year":"1995","journal-title":"Neuron"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB44","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1083\/jcb.129.3.551","article-title":"A nuclear localization domain in the hnRNP A1 protein","volume":"129","author":"Siomi","year":"1995","journal-title":"J. Cell Biol."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB45","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1038\/40153","article-title":"Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures","volume":"389","author":"Skinner","year":"1997","journal-title":"Nature"},{"key":"10.1016\/S0092-8674(00)81781-X_BIB46","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1093\/hmg\/6.3.487","article-title":"HIP-I","volume":"6","author":"Wanker","year":"1997","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB47","doi-asserted-by":"crossref","first-page":"9158","DOI":"10.1074\/jbc.273.15.9158","article-title":"Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract","volume":"273","author":"Wellington","year":"1998","journal-title":"J. Biol. Chem."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB48","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1038\/ng1297-404","article-title":"Huntingtin is required for neurogenesis and is not impaired by the Huntington\u2019s disease CAG expansion","volume":"17","author":"White","year":"1997","journal-title":"Nat. Genet."},{"key":"10.1016\/S0092-8674(00)81781-X_BIB49","unstructured":"Yi, H., and Hersch, S.M. (1997). Immunoelectron microscopic localization of receptors. In Receptor Localization: Laboratory Methods and Procedures, M. Ariano, ed. (New York: John Wiley & Sons), pp. 107\u2013127."}],"container-title":["Cell"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S009286740081781X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S009286740081781X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T10:39:48Z","timestamp":1758796788000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S009286740081781X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,10]]},"references-count":49,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1998,10]]}},"alternative-id":["S009286740081781X"],"URL":"https:\/\/doi.org\/10.1016\/s0092-8674(00)81781-x","relation":{},"ISSN":["0092-8674"],"issn-type":[{"value":"0092-8674","type":"print"}],"subject":[],"published":{"date-parts":[[1998,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Ataxin-1 Nuclear Localization and Aggregation","name":"articletitle","label":"Article Title"},{"value":"Cell","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/S0092-8674(00)81781-X","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"converted-article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 1998 Cell Press. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}