{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T01:51:09Z","timestamp":1793238669378,"version":"4.5.0"},"reference-count":119,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2009,3,1]],"date-time":"2009-03-01T00:00:00Z","timestamp":1235865600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2009,3,1]],"date-time":"2009-03-01T00:00:00Z","timestamp":1235865600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2009,3,1]],"date-time":"2009-03-01T00:00:00Z","timestamp":1235865600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2009,3,1]],"date-time":"2009-03-01T00:00:00Z","timestamp":1235865600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2009,3,1]],"date-time":"2009-03-01T00:00:00Z","timestamp":1235865600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2009,3,1]],"date-time":"2009-03-01T00:00:00Z","timestamp":1235865600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2009,3,1]],"date-time":"2009-03-01T00:00:00Z","timestamp":1235865600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS19620"],"award-info":[{"award-number":["NS19620"]}],"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":["NS28721"],"award-info":[{"award-number":["NS28721"]}],"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":[[2009,3]]},"DOI":"10.1016\/j.nbd.2008.10.009","type":"journal-article","created":{"date-parts":[[2008,11,11]],"date-time":"2008-11-11T03:05:37Z","timestamp":1226372737000},"page":"315-330","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":71,"title":["CAG repeat lengths \u2265 335 attenuate the phenotype in the R6\/2 Huntington's disease transgenic mouse"],"prefix":"10.1016","volume":"33","author":[{"given":"I.","family":"Dragatsis","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Goldowitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Del Mar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y.P.","family":"Deng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.A.","family":"Meade","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Z.","family":"Sun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Dietrich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Yue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Reiner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2008.10.009_bib1","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1111\/j.1469-1809.2006.00335.x","article-title":"The relationship between CAG repeat length and age of onset differs for Huntington's disease patients with juvenile onset or adult onset","volume":"71","author":"Andersen","year":"2006","journal-title":"Ann. Hum. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib2","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1098\/rstb.1999.0451","article-title":"Are there multiple pathways in the pathogenesis of Huntington's disease? Phil","volume":"354","author":"Aronin","year":"1999","journal-title":"Trans. R. Soc. Lond. B"},{"key":"10.1016\/j.nbd.2008.10.009_bib3","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/nature02998","article-title":"Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death","volume":"431","author":"Arrasate","year":"2004","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2008.10.009_bib4","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1212\/WNL.42.2.460","article-title":"Immunocytochemical localization of ubiquitin in inclusion body myositis allows its light-microscopic distinction from polymyositis","volume":"42","author":"Askanas","year":"1992","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2008.10.009_bib5","doi-asserted-by":"crossref","first-page":"2600","DOI":"10.1093\/hmg\/ddm217","article-title":"Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity","volume":"16","author":"Atwal","year":"2007","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib6","doi-asserted-by":"crossref","first-page":"25487","DOI":"10.1074\/jbc.M701600200","article-title":"The length dependence of the polyQ-mediated protein aggregation","volume":"282","author":"Barton","year":"2007","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2008.10.009_bib7","doi-asserted-by":"crossref","first-page":"3065","DOI":"10.1093\/hmg\/ddi340","article-title":"Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease","volume":"14","author":"Benn","year":"2005","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib8","doi-asserted-by":"crossref","first-page":"6809","DOI":"10.1073\/pnas.120166397","article-title":"Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice","volume":"97","author":"Bibb","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.nbd.2008.10.009_bib9","doi-asserted-by":"crossref","first-page":"5523","DOI":"10.1523\/JNEUROSCI.16-17-05523.1996","article-title":"Expression of normal and mutant huntingtin in the developing brain","volume":"16","author":"Bhide","year":"1996","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib10","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1093\/hmg\/ddi064","article-title":"Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin\u2013proteosome system impairment and inversely correlates with degree of nuclear inclusion formation","volume":"14","author":"Bowman","year":"2005","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib11","doi-asserted-by":"crossref","first-page":"13928","DOI":"10.1073\/pnas.1936025100","article-title":"Differential hydrophobicity drives self-assembly in Huntington's disease","volume":"100","author":"Burke","year":"2003","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.nbd.2008.10.009_bib12","doi-asserted-by":"crossref","first-page":"3248","DOI":"10.1523\/JNEUROSCI.19-08-03248.1999","article-title":"Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation","volume":"19","author":"Carter","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib13","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1002\/jnr.1244","article-title":"NMDA receptor function in mouse models of Huntington Disease","volume":"66","author":"Cepeda","year":"2001","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.nbd.2008.10.009_bib14","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1523\/JNEUROSCI.23-03-00961.2003","article-title":"Transient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington's disease","volume":"23","author":"Cepeda","year":"2003","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib15","doi-asserted-by":"crossref","first-page":"6480","DOI":"10.1073\/pnas.95.11.6480","article-title":"Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington's disease gene","volume":"95","author":"Cha","year":"1998","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.nbd.2008.10.009_bib16","doi-asserted-by":"crossref","first-page":"11884","DOI":"10.1073\/pnas.182276099","article-title":"Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation","volume":"99","author":"Chen","year":"2002","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.nbd.2008.10.009_bib17","first-page":"1527","article-title":"Reduced androgen receptor gene expression with first exon CAG repeat expansion","volume":"10","author":"Choong","year":"1996","journal-title":"Mol. Endocrinol."},{"key":"10.1016\/j.nbd.2008.10.009_bib18","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1038\/ng1503","article-title":"Polyglutamine expansion of huntingtin impairs its nuclear export","volume":"37","author":"Cornett","year":"2005","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2008.10.009_bib19","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\/j.nbd.2008.10.009_bib20","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.jchemneu.2004.02.005","article-title":"Differential loss of striatal projection systems in Huntington's disease, a quantitative immunohistochemical study","volume":"27","author":"Deng","year":"2004","journal-title":"J. Chem. Neuroanat."},{"key":"10.1016\/j.nbd.2008.10.009_bib21","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\/j.nbd.2008.10.009_bib22","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\/j.nbd.2008.10.009_bib23","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1038\/ng745","article-title":"Mutant protein in Huntington disease is resistant to proteolysis in affected brain","volume":"29","author":"Dyer","year":"2001","journal-title":"Nature Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib24","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/j.molmed.2005.01.001","article-title":"Gene dosage and pathogenesis of Parkinson's disease","volume":"11","author":"Eriksen","year":"2005","journal-title":"Trends Mol. Med."},{"key":"10.1016\/j.nbd.2008.10.009_bib25","doi-asserted-by":"crossref","first-page":"1463","DOI":"10.1093\/hmg\/7.9.1463","article-title":"Huntingtin interacts with a family of WW domain proteins","volume":"7","author":"Faber","year":"1998","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib26","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1093\/hmg\/10.2.145","article-title":"Abnormalities in the functioning of adipocytes from R6\/2 mice that are transgenic for the Huntington's disease mutation","volume":"10","author":"Fain","year":"2001","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib27","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1002\/ana.410390120","article-title":"Relationship between trinucleotide repeats and neuropathological changes in Huntington's disease","volume":"39","author":"Furtado","year":"1996","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib28","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1212\/WNL.57.4.708","article-title":"Juvenile Huntington's disease presenting as progressive myoclonic epilepsy","volume":"57","author":"Gambardella","year":"2001","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2008.10.009_bib29","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.cell.2004.06.018","article-title":"Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules","volume":"118","author":"Gauthier","year":"2004","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2008.10.009_bib30","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/S0306-4522(00)00008-7","article-title":"The pattern of neurodegeneration in Huntington's disease: a comparative study of cannabinoid, dopamine, adenosine and GABAA receptor alterations in the human basal ganglia in Huntington's disease","volume":"97","author":"Glass","year":"2000","journal-title":"Neuroscience"},{"key":"10.1016\/j.nbd.2008.10.009_bib31","doi-asserted-by":"crossref","first-page":"1513","DOI":"10.1126\/science.271.5255.1513","article-title":"Nucleocytoplasmic transport","volume":"271","author":"G\u00f6rlich","year":"1996","journal-title":"Science"},{"key":"10.1016\/j.nbd.2008.10.009_bib32","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1126\/science.3155875","article-title":"Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease","volume":"227","author":"Graveland","year":"1985","journal-title":"Science"},{"key":"10.1016\/j.nbd.2008.10.009_bib33","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1006\/exnr.2000.7626","article-title":"Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production","volume":"169","author":"Guidetti","year":"2001","journal-title":"Exper. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib34","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1001\/archneur.62.1.46","article-title":"Polyglutamine disease and transport problems. Deadly traffic jams on neuronal highways","volume":"62","author":"Gunawardena","year":"2005","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib35","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1523\/JNEUROSCI.19-07-02522.1999","article-title":"Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology","volume":"19","author":"Gutekunst","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib36","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1083\/jcb.141.5.1097","article-title":"The influence of huntingtin protein size on nuclear localization and cellular toxicity","volume":"141","author":"Hackam","year":"1998","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2008.10.009_bib37","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1111\/j.1399-0004.1998.tb02687.x","article-title":"The fatal attraction of polyglutamine-containing proteins","volume":"53","author":"Hackam","year":"1998","journal-title":"Clin. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib38","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1016\/S0361-9230(03)00185-0","article-title":"Standardization and statistical approaches to therapeutic trials in the R6\/2 mouse","volume":"61","author":"Hockly","year":"2005","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.nbd.2008.10.009_bib39","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0896-6273(00)80764-3","article-title":"A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration","volume":"23","author":"Hodgson","year":"1999","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2008.10.009_bib40","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 the HD chromosome","volume":"72","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2008.10.009_bib41","doi-asserted-by":"crossref","first-page":"649","DOI":"10.2337\/diabetes.48.3.649","article-title":"Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes","volume":"48","author":"Hurlbert","year":"1999","journal-title":"Diabetes"},{"key":"10.1016\/j.nbd.2008.10.009_bib42","doi-asserted-by":"crossref","first-page":"1621","DOI":"10.1093\/hmg\/ddg163","article-title":"Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context","volume":"12","author":"Jackson","year":"2003","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib43","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1111\/j.1365-2990.2003.00545.x","article-title":"Frontotemporal lobar degeneration and ubiquitin immunohistochemistry","volume":"30","author":"Josephs","year":"2004","journal-title":"Neuropath. Appl. Neurobiol."},{"key":"10.1016\/j.nbd.2008.10.009_bib44","doi-asserted-by":"crossref","first-page":"3359","DOI":"10.1093\/hmg\/ddg352","article-title":"Dramatic tissue-specific mutation length increases are an early molecular event in Huntington's disease pathogenesis","volume":"12","author":"Kennedy","year":"2003","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib45","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1016\/S0896-6273(00)80581-4","article-title":"Neuronal intranuclear inclusions in polyglutamine diseases: nuclear weapons or nuclear fallout","volume":"21","author":"Kim","year":"1998","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2008.10.009_bib46","doi-asserted-by":"crossref","first-page":"2667","DOI":"10.1152\/jn.2001.86.6.2667","article-title":"Electrophysiological and morphological changes in striatal spiny neurons in R6\/2 Huntington's disease transgenic mice","volume":"86","author":"Klapstein","year":"2001","journal-title":"J. Neurophysiol."},{"key":"10.1016\/j.nbd.2008.10.009_bib47","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/S0092-8674(00)81781-X","article-title":"Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice","volume":"95","author":"Klement","year":"1998","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2008.10.009_bib48","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1002\/1531-8249(199912)46:6<842::AID-ANA6>3.0.CO;2-O","article-title":"Huntingtin aggregates may not predict neuronal death in Huntington's disease","volume":"46","author":"Kuemmerle","year":"1999","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib49","doi-asserted-by":"crossref","first-page":"9112","DOI":"10.1523\/JNEUROSCI.21-23-09112.2001","article-title":"Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease","volume":"21","author":"Laforet","year":"2001","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib50","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/S0896-6273(03)00328-3","article-title":"Polyglutamines placed into cortex","volume":"38","author":"La Spada","year":"2003","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2008.10.009_bib51","doi-asserted-by":"crossref","first-page":"8289","DOI":"10.1523\/JNEUROSCI.1990-04.2004","article-title":"Evidence for differential cortical input to direct pathway versus indirect pathway striatal projection neurons in rats","volume":"24","author":"Lei","year":"2004","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib52","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1093\/hmg\/7.5.777","article-title":"Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats","volume":"7","author":"Li","year":"1998","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib53","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.1093\/hmg\/8.7.1227","article-title":"Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice","volume":"8","author":"Li","year":"1999","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib54","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. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib55","doi-asserted-by":"crossref","first-page":"10428","DOI":"10.1523\/JNEUROSCI.19-23-10428.1999","article-title":"Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation","volume":"19","author":"Lione","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib56","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1093\/hmg\/9.9.1259","article-title":"Decreased expression of striatal signaling genes in a mouse model of Huntington's disease","volume":"9","author":"Luthi-Carter","year":"2000","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib57","doi-asserted-by":"crossref","first-page":"1911","DOI":"10.1093\/hmg\/11.17.1911","article-title":"Dysregulation of gene expression in the R6\/2 model of polyglutamine disease: parallel changes in muscle and brain","volume":"11","author":"Luthi-Carter","year":"2002","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib58","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\/j.nbd.2008.10.009_bib59","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1097\/00005072-199902000-00003","article-title":"Distribution of inclusions in neuronal nuclei and dystrophic neurites in Huntington disease brain","volume":"58","author":"Maat-Schieman","year":"1999","journal-title":"J. Neuropathol. Exper. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib60","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1038\/ng0298-150","article-title":"Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates","volume":"18","author":"Martindale","year":"1998","journal-title":"Nature Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib61","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1002\/cne.10295","article-title":"Localization of neuronal intranuclear inclusions (NIIs) in the striatum and cortex of the Bates R6\/2 transgenic mouse","volume":"449","author":"Meade","year":"2002","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib62","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1006\/exnr.1999.7327","article-title":"Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease","volume":"162","author":"Menalled","year":"2000","journal-title":"Exper. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib63","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1038\/sj.cdd.4401093","article-title":"`Tissue' transglutaminase ablation reduces neuronal death and prolongs survival in a mouse model of Huntington's disease","volume":"9","author":"Mastroberardino","year":"2002","journal-title":"Cell Death Differ."},{"key":"10.1016\/j.nbd.2008.10.009_bib64","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/j.nbd.2006.09.016","article-title":"Cardiac dysfunction in the R6\/2 mouse model of Huntington's disease","volume":"25","author":"Mihm","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2008.10.009_bib65","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1034\/j.1399-0004.2003.00108.x","article-title":"XL PCR for the detection of large trinucleotide expansions in juvenile Huntington's disease","volume":"64","author":"Milunsky","year":"2003","journal-title":"Clin. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib66","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1023\/A:1010887421592","article-title":"Progressive formation of inclusions in the striatum and hippocampus of mice transgenic for the human Huntington's disease mutation","volume":"29","author":"Morton","year":"2000","journal-title":"J. Neurocytol."},{"key":"10.1016\/j.nbd.2008.10.009_bib67","doi-asserted-by":"crossref","unstructured":"Morton, A.J., Glynn, D., Leavens, W., Zheng, Z., Skepper, J., Wigth, J., Fyfe, I., 2008. Paradoxical delay in the onset of disease caused by superlong CAG repeat expansions unmasks neurodegeneration in R6\/2 mice. Hereditary Disease Foundation Symposium: HD2008: \u201cThe Milton Wexler Celebration of Life\u201d. Abst# 26.","DOI":"10.1016\/j.nbd.2008.11.015"},{"key":"10.1016\/j.nbd.2008.10.009_bib68","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1242\/dev.116.1.201","article-title":"NeuN, a neuronal specific nuclear protein in vertebrates","volume":"116","author":"Mullen","year":"1992","journal-title":"Development"},{"key":"10.1016\/j.nbd.2008.10.009_bib69","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1002\/ajmg.a.30891","article-title":"Juvenile onset Huntington disease resulting from a very large maternal expansion","volume":"137A","author":"Nahhas","year":"2005","journal-title":"Am. J. Med. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib70","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1212\/WNL.52.2.392","article-title":"Analysis of a very large trinucleotide repeat in a patient with juvenile Huntington's disease","volume":"52","author":"Nance","year":"1999","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2008.10.009_bib71","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1136\/jmg.36.10.739","article-title":"A molecular investigation of true dominance in Huntington's disease","volume":"36","author":"Narain","year":"1999","journal-title":"J. Med. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib72","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1046\/j.1471-4159.2003.02009.x","article-title":"Inclusion formation in Huntington's disease R6\/2 mouse muscle cultures","volume":"87","author":"Orth","year":"2003","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2008.10.009_bib74","doi-asserted-by":"crossref","first-page":"1380","DOI":"10.1002\/mds.20557","article-title":"Case of maternally transmitted juvenile Huntington's disease with a very large trinucleotide repeat","volume":"20","author":"Papapetropoulos","year":"2005","journal-title":"Movement Dis."},{"key":"10.1016\/j.nbd.2008.10.009_bib75","doi-asserted-by":"crossref","first-page":"2441","DOI":"10.1093\/brain\/awh265","article-title":"Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy","volume":"127","author":"Paviour","year":"2004","journal-title":"Brain"},{"key":"10.1016\/j.nbd.2008.10.009_bib76","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1002\/ana.410410521","article-title":"CAG repeat number governs the development rate of pathology in Huntington's disease","volume":"41","author":"Penney","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib77","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1523\/JNEUROSCI.1409-03.2004","article-title":"Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction","volume":"24","author":"Qin","year":"2004","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib78","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1385\/MN:28:3:259","article-title":"Wild-type huntingtin plays a role in brain development and neuronal survival","volume":"28","author":"Reiner","year":"2003","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.nbd.2008.10.009_bib79","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1002\/cne.21515","article-title":"R6\/2 Neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice","volume":"505","author":"Reiner","year":"2007","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib80","doi-asserted-by":"crossref","first-page":"3092","DOI":"10.1111\/j.1460-9568.2004.03783.x","article-title":"Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation","volume":"20","author":"Ribchester","year":"2004","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib81","doi-asserted-by":"crossref","first-page":"3705","DOI":"10.1523\/JNEUROSCI.20-10-03705.2000","article-title":"Wild-type huntingtin protects from apoptosis upstream of caspase-3","volume":"20","author":"Rigamonti","year":"2000","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib82","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.1212\/01.wnl.0000204230.16619.d9","article-title":"The association of CAG repeat length with clinical progression in Huntington's disease","volume":"66","author":"Rosenblatt","year":"2006","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2008.10.009_bib83","doi-asserted-by":"crossref","first-page":"S10","DOI":"10.1038\/nm1066","article-title":"Protein aggregation and neurodegenerative disease","volume":"10","author":"Ross","year":"2004","journal-title":"Nature Med."},{"key":"10.1016\/j.nbd.2008.10.009_bib84","doi-asserted-by":"crossref","first-page":"2425","DOI":"10.1093\/hmg\/10.21.2425","article-title":"Centrosome disorganization in fibroblast cultures derived from R6\/2 Huntington's disease (HD) transgenic mice and HD patients","volume":"10","author":"Sathasivam","year":"2001","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib85","first-page":"373","article-title":"Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders","volume":"421","author":"Sanchez","year":"2003","journal-title":"Science"},{"key":"10.1016\/j.nbd.2008.10.009_bib86","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1097\/00005072-199902000-00006","article-title":"Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease","volume":"58","author":"Sapp","year":"1999","journal-title":"J. Neuropathol. Exper. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib87","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\/j.nbd.2008.10.009_bib88","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1093\/hmg\/8.3.397","article-title":"Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin","volume":"8","author":"Schilling","year":"1999","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib89","doi-asserted-by":"crossref","first-page":"1599","DOI":"10.1093\/hmg\/ddh175","article-title":"Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice","volume":"13","author":"Schilling","year":"2004","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib90","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/s00431-004-1537-3","article-title":"Early onset Huntington disease: a neuronal degeneration syndrome","volume":"163","author":"Seneca","year":"2004","journal-title":"Eur. J. Pediat."},{"key":"10.1016\/j.nbd.2008.10.009_bib91","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1093\/hmg\/8.5.763","article-title":"A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice","volume":"8","author":"Shelbourne","year":"1999","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib92","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1093\/hmg\/ddm054","article-title":"Triplet repeat mutation length gains correlate with cell-type specific vulnerability in Huntington disease brain","volume":"16","author":"Shelbourne","year":"2007","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib93","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1006\/exnr.1998.7005","article-title":"Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein","volume":"156","author":"Sieradzan","year":"1999","journal-title":"Exper. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib94","doi-asserted-by":"crossref","first-page":"1953","DOI":"10.1093\/hmg\/11.17.1953","article-title":"Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses","volume":"11","author":"Sipione","year":"2002","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib95","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0092-8674(00)81743-2","article-title":"Nuclear inclusion in glutamine repeat disorders: are they pernicious, coincidental or beneficial?","volume":"95","author":"Sisodia","year":"1998","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2008.10.009_bib96","doi-asserted-by":"crossref","first-page":"11402","DOI":"10.1073\/pnas.0503634102","article-title":"Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions","volume":"102","author":"Slow","year":"2005","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.nbd.2008.10.009_bib97","doi-asserted-by":"crossref","first-page":"6469","DOI":"10.1073\/pnas.95.11.6469","article-title":"Alpha-synuclein in filamentous inclusions of Lewy bodies from Parkinson's disease and dementia with Lewy bodies","volume":"95","author":"Spillantini","year":"1998","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.nbd.2008.10.009_bib98","doi-asserted-by":"crossref","first-page":"2799","DOI":"10.1111\/j.0953-816X.2004.03374.x","article-title":"Dendritic spine pathology and deficits in experience-dependent dendritic plasticity in R6\/1 Huntington's disease transgenic mice","volume":"19","author":"Spires","year":"2004","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib99","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1002\/1096-8628(20001211)95:4<366::AID-AJMG13>3.0.CO;2-2","article-title":"Family and molecular data for a fine analysis of age at onset in Huntington Disease","volume":"95","author":"Squitieri","year":"2000","journal-title":"Am. J. Med. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib100","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1016\/j.mad.2005.09.012","article-title":"Juvenile Huntington's disease: does a dosage-effect pathogenic mechanism differ from the classical adult disease? Mech","volume":"127","author":"Squitieri","year":"2006","journal-title":"Aging Devel."},{"key":"10.1016\/j.nbd.2008.10.009_bib101","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1002\/cne.20680","article-title":"Chronology of behavioral symptoms and neuropathological sequela in R6\/2 Huntington 's disease transgenic mice","volume":"490","author":"Stack","year":"2005","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib102","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1093\/hmg\/2.10.1547","article-title":"Correlation between the onset age of Huntington's disease and length of the trinucleotide repeat in IT15","volume":"2","author":"Stine","year":"1993","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib103","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1006\/nbdi.2002.0554","article-title":"Differential changes in striatal projection neurons in R6\/2 mice transgenic for Huntington's disease","volume":"11","author":"Sun","year":"2002","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2008.10.009_bib104","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1016\/S1534-5807(03)00162-X","article-title":"Peering through the pore: nuclear pore complex structure, assembly, and function","volume":"4","author":"Suntharalingam","year":"2003","journal-title":"Dev. Cell."},{"key":"10.1016\/j.nbd.2008.10.009_bib105","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1093\/hmg\/ddm311","article-title":"Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic","volume":"17","author":"Takahashi","year":"2008","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib106","doi-asserted-by":"crossref","first-page":"4237","DOI":"10.1523\/JNEUROSCI.21-12-04237.2001","article-title":"AMPA\/kainate receptor activation mediates hypoxic oligodendrocyte death and axonal injury in cerebral white matter","volume":"21","author":"Tekkok","year":"2001","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib107","doi-asserted-by":"crossref","first-page":"1412","DOI":"10.1126\/science.1142204","article-title":"Crossing the nuclear envelope: hierarchical regulation of nucleocytoplasmic transport","volume":"318","author":"Terry","year":"2007","journal-title":"Science"},{"key":"10.1016\/j.nbd.2008.10.009_bib108","doi-asserted-by":"crossref","first-page":"12171","DOI":"10.1073\/pnas.2034961100","article-title":"Microtubule destabilization and nuclear entry are sequential steps leading to toxicity in Huntington's disease","volume":"100","author":"Trushina","year":"2003","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.nbd.2008.10.009_bib109","doi-asserted-by":"crossref","first-page":"8195","DOI":"10.1128\/MCB.24.18.8195-8209.2004","article-title":"Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro","volume":"24","author":"Trushina","year":"2004","journal-title":"Mol. Cell Biol."},{"key":"10.1016\/j.nbd.2008.10.009_bib110","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1093\/hmg\/9.4.503","article-title":"Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice","volume":"9","author":"Wheeler","year":"2000","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib111","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1006\/exnr.1998.6832","article-title":"Wild-type and mutant huntingtin function in vesicle trafficking in the secretory and endocytotic pathways","volume":"152","author":"Velier","year":"1998","journal-title":"Exper. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib112","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1083\/jcb.200710158","article-title":"Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin","volume":"181","author":"Wang","year":"2008","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2008.10.009_bib113","doi-asserted-by":"crossref","first-page":"2789","DOI":"10.1093\/hmg\/ddg300","article-title":"Regional differences of somatic CAG repeat instability do not account for selective neuronal vulnerability in a knock-in mouse model of SCA1","volume":"12","author":"Watase","year":"2003","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib114","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1111\/j.1471-4159.2007.05032.x","article-title":"Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease","volume":"104","author":"Weiss","year":"2008","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2008.10.009_bib120","doi-asserted-by":"crossref","first-page":"1068","DOI":"10.1177\/7010.2006.00244","article-title":"Huntington Disease in a 9-year-old boy: Clinical course and neuropathologic examination","volume":"21","author":"Wojaczynska-Stanek","year":"2006","journal-title":"J. Child. Neurol."},{"key":"10.1016\/j.nbd.2008.10.009_bib115","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1177\/44.10.8813082","article-title":"NeuN: a useful neuronal marker for diagnostic histopathology","volume":"44","author":"Wolf","year":"1996","journal-title":"J. Histochem. Cytochem."},{"key":"10.1016\/j.nbd.2008.10.009_bib116","doi-asserted-by":"crossref","first-page":"2905","DOI":"10.1093\/hmg\/11.23.2905","article-title":"Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells","volume":"11","author":"Yang","year":"2002","journal-title":"Hum. Mol. Gen."},{"key":"10.1016\/j.nbd.2008.10.009_bib117","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's disease","volume":"23","author":"Yu","year":"2003","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2008.10.009_bib118","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1111\/j.1469-7580.2006.00573.x","article-title":"Total number of neurons in the habenular nuclei of the rat epithalamus, a stereological study","volume":"208","author":"Zhang","year":"2006","journal-title":"J. Anat"},{"key":"10.1016\/j.nbd.2008.10.009_bib119","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1146\/annurev.neuro.23.1.217","article-title":"Glutamine repeats and neurodegeneration","volume":"23","author":"Zoghbi","year":"2000","journal-title":"Ann. Rev. Neurosci."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996108002568?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996108002568?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T05:04:43Z","timestamp":1760850283000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996108002568"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,3]]},"references-count":119,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2009,3]]}},"alternative-id":["S0969996108002568"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2008.10.009","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2009,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"CAG repeat lengths \u2265335 attenuate the phenotype in the R6\/2 Huntington's disease transgenic mouse","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2008.10.009","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2008 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}