{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T01:49:54Z","timestamp":1791078594216,"version":"4.1.0"},"reference-count":96,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2011,4,1]],"date-time":"2011-04-01T00:00:00Z","timestamp":1301616000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2011,4,1]],"date-time":"2011-04-01T00:00:00Z","timestamp":1301616000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2011,4,1]],"date-time":"2011-04-01T00:00:00Z","timestamp":1301616000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2011,4,1]],"date-time":"2011-04-01T00:00:00Z","timestamp":1301616000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2011,4,1]],"date-time":"2011-04-01T00:00:00Z","timestamp":1301616000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2011,4,1]],"date-time":"2011-04-01T00:00:00Z","timestamp":1301616000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2011,4,1]],"date-time":"2011-04-01T00:00:00Z","timestamp":1301616000000},"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":["1R01 NS48588-01"],"award-info":[{"award-number":["1R01 NS48588-01"]}],"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":["T32AG00196"],"award-info":[{"award-number":["T32AG00196"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Experimental Neurology"],"published-print":{"date-parts":[[2011,4]]},"DOI":"10.1016\/j.expneurol.2010.12.017","type":"journal-article","created":{"date-parts":[[2010,12,29]],"date-time":"2010-12-29T04:13:28Z","timestamp":1293596008000},"page":"173-182","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":36,"title":["Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats"],"prefix":"10.1016","volume":"228","author":[{"given":"Aaron C.","family":"Rising","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jia","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aaron","family":"Carlson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent V.","family":"Napoli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eileen M.","family":"Denovan-Wright","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald J.","family":"Mandel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.expneurol.2010.12.017_bb0005","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1002\/ana.410300406","article-title":"Preproenkephalin messenger RNA-containing neurons in striatum of patients with symptomatic and presymptomatic Huntington's disease: an in situ hybridization study","volume":"30","author":"Albin","year":"1991","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0010","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1002\/ana.410420213","article-title":"Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease","volume":"42","author":"Augood","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0015","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1006\/nbdi.1998.0168","article-title":"Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length","volume":"4","author":"Becher","year":"1998","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0020","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1016\/S0028-3908(01)00072-7","article-title":"Behavioral and neurochemical changes following predatory stress in mice","volume":"41","author":"Belzung","year":"2001","journal-title":"Neuropharmacology"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0025","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1124\/pr.58.3.5","article-title":"Cyclic nucleotide phosphodiesterases: molecular regulation to clinical use","volume":"58","author":"Bender","year":"2006","journal-title":"Pharmacol. Rev."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0030","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. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0035","doi-asserted-by":"crossref","first-page":"1769","DOI":"10.1111\/j.1471-4159.1991.tb06379.x","article-title":"Cannabinoid receptor-regulated cyclic AMP accumulation in the rat striatum","volume":"57","author":"Bidaut-Russell","year":"1991","journal-title":"J. Neurochem."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0040","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.expneurol.2010.12.017_bb0045","doi-asserted-by":"crossref","first-page":"1939","DOI":"10.1093\/hmg\/11.17.1939","article-title":"Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease","volume":"11","author":"Chan","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0050","first-page":"219","article-title":"Behavioral changes in Huntington disease","volume":"14","author":"Craufurd","year":"2001","journal-title":"Neuropsychiatry Neuropsychol. Behav. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0055","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.expneurol.2010.12.017_bb0060","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1016\/S0306-4522(00)00157-3","article-title":"Cannabinoid receptor messenger RNA levels decrease in a subset of neurons of the lateral striatum, cortex and hippocampus of transgenic Huntington's disease mice","volume":"98","author":"Denovan-Wright","year":"2000","journal-title":"Neuroscience"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0065","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/j.bbr.2009.09.014","article-title":"Distinct contributions of the hippocampus and medial prefrontal cortex to the \u201cwhat\u2013where\u2013when\u201d components of episodic-like memory in mice","volume":"215","author":"Devito","year":"2010","journal-title":"Behav. Brain Res."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0070","doi-asserted-by":"crossref","first-page":"17204","DOI":"10.1073\/pnas.0708285104","article-title":"Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits","volume":"104","author":"DiFiglia","year":"2007","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0075","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.bbr.2006.12.004","article-title":"Sex differences in behavior and striatal ascorbate release in the 140 CAG knock-in mouse model of Huntington's disease","volume":"178","author":"Dorner","year":"2007","journal-title":"Behav. Brain Res."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0080","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.neuroscience.2009.06.014","article-title":"Altered CB1 receptor and endocannabinoid levels precede motor symptom onset in a transgenic mouse model of Huntington's disease","volume":"163","author":"Dowie","year":"2009","journal-title":"Neuroscience"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0085","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1002\/ana.21569","article-title":"Sustained effects of nonallele-specific Huntingtin silencing","volume":"65","author":"Drouet","year":"2009","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0090","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1002\/ana.20684","article-title":"Preclinical Huntington's disease: compensatory brain responses during learning","volume":"59","author":"Feigin","year":"2006","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0095","first-page":"168","article-title":"Huntington disease in Maryland: clinical aspects of racial variation","volume":"41","author":"Folstein","year":"1987","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0100","doi-asserted-by":"crossref","first-page":"1032","DOI":"10.1212\/01.WNL.0000154530.72969.11","article-title":"Normative estimates of cross-sectional and longitudinal brain volume decline in aging and AD","volume":"64","author":"Fotenos","year":"2005","journal-title":"Neurology"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0105","doi-asserted-by":"crossref","first-page":"18438","DOI":"10.1074\/jbc.274.26.18438","article-title":"Cloning and characterization of a novel human phosphodiesterase that hydrolyzes both cAMP and cGMP (PDE10A)","volume":"274","author":"Fujishige","year":"1999","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0110","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1037\/0894-4105.11.2.272","article-title":"Intact mirror-tracing and impaired rotary-pursuit skill learning in patients with Huntington's disease: evidence for dissociable memory systems in skill learning","volume":"11","author":"Gabrieli","year":"1997","journal-title":"Neuropsychology"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0115","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1016\/j.parkreldis.2007.11.009","article-title":"Implicit and explicit aspects of sequence learning in pre-symptomatic Huntington's disease","volume":"14","author":"Ghilardi","year":"2008","journal-title":"Parkinsonism Relat. Disord."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0120","doi-asserted-by":"crossref","first-page":"5327","DOI":"10.1523\/JNEUROSCI.17-14-05327.1997","article-title":"Concurrent stimulation of cannabinoid CB1 and dopamine D2 receptors augments cAMP accumulation in striatal neurons: evidence for a Gs linkage to the CB1 receptor","volume":"17","author":"Glass","year":"1997","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0125","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.parkreldis.2008.05.010","article-title":"Prefrontal cortex volume reduction on MRI in preclinical Huntington's disease relates to visuomotor performance and CAG number","volume":"15","author":"Gomez-Anson","year":"2009","journal-title":"Parkinsonism Relat. Disord."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0130","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.expneurol.2010.12.017_bb0135","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.expneurol.2010.12.017_bb0140","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1006\/exnr.1998.6919","article-title":"Regional specificity of brain atrophy in Huntington's disease","volume":"154","author":"Halliday","year":"1998","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0145","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1136\/bmj.307.6901.397","article-title":"Clinical consequences of isolating the gene for Huntington's disease","volume":"307","author":"Harper","year":"1993","journal-title":"BMJ"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0150","doi-asserted-by":"crossref","first-page":"5820","DOI":"10.1073\/pnas.0501507102","article-title":"RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model","volume":"102","author":"Harper","year":"2005","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0155","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1016\/j.neuroscience.2003.11.009","article-title":"Striatal phosphodiesterase mRNA and protein levels are reduced in Huntington's disease transgenic mice prior to the onset of motor symptoms","volume":"123","author":"Hebb","year":"2004","journal-title":"Neuroscience"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0160","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/j.euroneuro.2007.08.002","article-title":"Phosphodiesterase 10A inhibition is associated with locomotor and cognitive deficits and increased anxiety in mice","volume":"18","author":"Hebb","year":"2008","journal-title":"Eur. Neuropsychopharmacol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0165","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1016\/0006-8993(91)90970-7","article-title":"Neuronal localization of cannabinoid receptors in the basal ganglia of the rat","volume":"547","author":"Herkenham","year":"1991","journal-title":"Brain Res."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0170","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1016\/j.neuroscience.2008.08.041","article-title":"Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice","volume":"157","author":"Hickey","year":"2008","journal-title":"Neuroscience"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0175","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1136\/jnnp.2009.190702","article-title":"The progression of regional atrophy in premanifest and early Huntington's disease: a longitudinal voxel-based morphometry study","volume":"81","author":"Hobbs","year":"2010","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0180","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.3174\/ajnr.A2018","article-title":"Onset and progression of pathologic atrophy in Huntington disease: a longitudinal MR imaging study","volume":"31","author":"Hobbs","year":"2010","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0185","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.expneurol.2010.12.017_bb0190","doi-asserted-by":"crossref","first-page":"3351","DOI":"10.1111\/j.1460-9568.2004.03796.x","article-title":"Mutant huntingtin affects the rate of transcription of striatum-specific isoforms of phosphodiesterase 10A","volume":"20","author":"Hu","year":"2004","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0195","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1001\/archneur.1983.04050120041005","article-title":"Development of neuropsychological deficits in Huntington's disease","volume":"40","author":"Josiassen","year":"1983","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0200","doi-asserted-by":"crossref","first-page":"1845","DOI":"10.1093\/hmg\/ddm133","article-title":"Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage","volume":"16","author":"Kuhn","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0205","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1016\/j.neuropharm.2010.05.004","article-title":"Quantitative comparison of phosphodiesterase mRNA distribution in human brain and peripheral tissues","volume":"59","author":"Lakics","year":"2010","journal-title":"Neuropharmacology"},{"issue":"Pt 5","key":"10.1016\/j.expneurol.2010.12.017_bb0210","doi-asserted-by":"crossref","first-page":"1633","DOI":"10.1093\/brain\/119.5.1633","article-title":"Executive and mnemonic functions in early Huntington's disease","volume":"119","author":"Lawrence","year":"1996","journal-title":"Brain"},{"issue":"Pt 7","key":"10.1016\/j.expneurol.2010.12.017_bb0215","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1093\/brain\/121.7.1329","article-title":"Evidence for specific cognitive deficits in preclinical Huntington's disease","volume":"121","author":"Lawrence","year":"1998","journal-title":"Brain"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0220","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1073\/pnas.87.1.230","article-title":"Dopamine receptor gene expression by enkephalin neurons in rat forebrain","volume":"87","author":"Le Moine","year":"1990","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0225","doi-asserted-by":"crossref","first-page":"4205","DOI":"10.1073\/pnas.88.10.4205","article-title":"Phenotypical characterization of the rat striatal neurons expressing the D1 dopamine receptor gene","volume":"88","author":"Le Moine","year":"1991","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0230","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1002\/(SICI)1097-4547(19991115)58:4<515::AID-JNR5>3.0.CO;2-F","article-title":"Enhanced sensitivity to N-methyl-d-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease","volume":"58","author":"Levine","year":"1999","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0235","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.expneurol.2010.12.017_bb0240","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/S0378-1119(99)00171-7","article-title":"Isolation and characterization of PDE10A, a novel human 3\u2032, 5\u2032-cyclic nucleotide phosphodiesterase","volume":"234","author":"Loughney","year":"1999","journal-title":"Gene"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0245","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. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0250","doi-asserted-by":"crossref","first-page":"1927","DOI":"10.1093\/hmg\/11.17.1927","article-title":"Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects","volume":"11","author":"Luthi-Carter","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0255","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.expneurol.2010.12.017_bb0260","doi-asserted-by":"crossref","first-page":"4373","DOI":"10.1093\/hmg\/ddq358","article-title":"The Huntington's disease mutation impairs Huntingtin's role in the transport of NF-{kappa}B from the synapse to the nucleus","volume":"19","author":"Marcora","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0265","doi-asserted-by":"crossref","first-page":"9578","DOI":"10.1073\/pnas.1133382100","article-title":"Stimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2","volume":"100","author":"Marcora","year":"2003","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0270","doi-asserted-by":"crossref","first-page":"4909","DOI":"10.1111\/j.1432-1033.2004.04460.x","article-title":"Structure, expression and regulation of the cannabinoid receptor gene (CB1) in Huntington's disease transgenic mice","volume":"271","author":"McCaw","year":"2004","journal-title":"Eur. J. Biochem."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0275","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":"Exp. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0280","doi-asserted-by":"crossref","first-page":"8266","DOI":"10.1523\/JNEUROSCI.22-18-08266.2002","article-title":"Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice","volume":"22","author":"Menalled","year":"2002","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0285","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1002\/cne.10776","article-title":"Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats","volume":"465","author":"Menalled","year":"2003","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0290","doi-asserted-by":"crossref","first-page":"2609","DOI":"10.1523\/JNEUROSCI.12-07-02609.1992","article-title":"Dynamic regulation of NGFI-A (zif268, egr1) gene expression in the striatum","volume":"12","author":"Moratalla","year":"1992","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0295","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.expneurol.2010.12.017_bb0300","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/S0924-9338(01)00604-6","article-title":"Huntington's disease: a review of the literature on prevalence and treatment of neuropsychiatric phenomena","volume":"16","author":"Naarding","year":"2001","journal-title":"Eur. Psychiatry"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0305","doi-asserted-by":"crossref","first-page":"8147","DOI":"10.1523\/JNEUROSCI.17-21-08147.1997","article-title":"Bidirectional regulation of DARPP-32 phosphorylation by dopamine","volume":"17","author":"Nishi","year":"1997","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0310","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.jns.2005.07.007","article-title":"Executive dysfunction in early stages of Huntington's disease is associated with striatal and insular atrophy: a neuropsychological and voxel-based morphometric study","volume":"239","author":"Peinemann","year":"2005","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0315","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1016\/j.cub.2009.03.030","article-title":"Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients","volume":"19","author":"Pfister","year":"2009","journal-title":"Curr. Biol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0320","doi-asserted-by":"crossref","first-page":"1491","DOI":"10.1002\/mds.21971","article-title":"The current clinical management of Huntington's disease","volume":"23","author":"Phillips","year":"2008","journal-title":"Mov. Disord."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0325","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1007\/s003359900820","article-title":"Genomic structure and chromosome location of the murine PDE1B phosphodiesterase gene","volume":"9","author":"Reed","year":"1998","journal-title":"Mamm. Genome"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0330","doi-asserted-by":"crossref","first-page":"5733","DOI":"10.1073\/pnas.85.15.5733","article-title":"Differential loss of striatal projection neurons in Huntington disease","volume":"85","author":"Reiner","year":"1988","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0335","doi-asserted-by":"crossref","first-page":"3295","DOI":"10.1523\/JNEUROSCI.23-08-03295.2003","article-title":"Longitudinal magnetic resonance imaging studies of older adults: a shrinking brain","volume":"23","author":"Resnick","year":"2003","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0340","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1002\/ana.410370309","article-title":"Reduced expression of preproenkephalin in striatal neurons from Huntington's disease patients","volume":"37","author":"Richfield","year":"1995","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0345","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1037\/0894-4105.21.1.31","article-title":"Early cognitive deficits in Swedish gene carriers of Huntington's disease","volume":"21","author":"Robins Wahlin","year":"2007","journal-title":"Neuropsychology"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0350","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1016\/j.ymthe.2005.05.006","article-title":"Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6\/1 Huntington's disease transgenic mice","volume":"12","author":"Rodriguez-Lebron","year":"2005","journal-title":"Mol. Ther."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0355","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1212\/01.wnl.0000174432.87383.87","article-title":"Regional cortical thinning in preclinical Huntington disease and its relationship to cognition","volume":"65","author":"Rosas","year":"2005","journal-title":"Neurology"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0360","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1093\/brain\/awn025","article-title":"Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity","volume":"131","author":"Rosas","year":"2008","journal-title":"Brain"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0365","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1590\/S0100-879X2006000800016","article-title":"Striatal and extrastriatal atrophy in Huntington's disease and its relationship with length of the CAG repeat","volume":"39","author":"Ruocco","year":"2006","journal-title":"Braz. J. Med. Biol. Res."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0370","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1001\/archneur.60.7.989","article-title":"A longitudinal study of brain volume changes in normal aging using serial registered magnetic resonance imaging","volume":"60","author":"Scahill","year":"2003","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0375","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.molcel.2004.06.029","article-title":"Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation","volume":"15","author":"Schaffar","year":"2004","journal-title":"Mol. Cell"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0380","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. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0385","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1203\/01.PDR.0000180553.23507.31","article-title":"Locomotor effects of a D1R agonist are DARPP-32 dependent in adult but not weanling mice","volume":"58","author":"Scott","year":"2005","journal-title":"Pediatr. Res."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0390","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1093\/hmg\/ddg169","article-title":"Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease","volume":"12","author":"Slow","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0395","doi-asserted-by":"crossref","first-page":"6763","DOI":"10.1073\/pnas.100110097","article-title":"The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription","volume":"97","author":"Steffan","year":"2000","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0400","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1038\/294366a0","article-title":"Opposing roles for D-1 and D-2 dopamine receptors in efflux of cyclic AMP from rat neostriatum","volume":"294","author":"Stoof","year":"1981","journal-title":"Nature"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0405","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/0014-2999(86)90358-4","article-title":"D-2 receptor stimulation inhibits cyclic AMP formation brought about by D-1 receptor stimulation in rat neostriatum but not nucleus accumbens","volume":"129","author":"Stoof","year":"1986","journal-title":"Eur. J. Pharmacol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0410","doi-asserted-by":"crossref","first-page":"4988","DOI":"10.1074\/jbc.M310226200","article-title":"Decreased cAMP response element-mediated transcription: an early event in exon 1 and full-length cell models of Huntington's disease that contributes to polyglutamine pathogenesis","volume":"279","author":"Sugars","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0415","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1097\/00001756-200502280-00019","article-title":"Polyglutamine represses cAMP-responsive-element-mediated transcription without aggregate formation","volume":"16","author":"Takahashi","year":"2005","journal-title":"NeuroReport"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0420","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/1471-2202-3-15","article-title":"The predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB\/Rel\/dorsal family transcription factor","volume":"3","author":"Takano","year":"2002","journal-title":"BMC Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0425","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's disease chromosomes","volume":"72","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0430","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1176\/jnp.14.1.37","article-title":"Behavior in Huntington's disease: dissociating cognition-based and mood-based changes","volume":"14","author":"Thompson","year":"2002","journal-title":"J. Neuropsychiatry Clin. Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0435","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/S0306-4522(97)00436-3","article-title":"Immunohistochemical distribution of cannabinoid CB1 receptors in the rat central nervous system","volume":"83","author":"Tsou","year":"1998","journal-title":"Neuroscience"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0440","doi-asserted-by":"crossref","first-page":"3751","DOI":"10.1097\/00001756-200011270-00032","article-title":"N-Acetylaspartate and DARPP-32 levels decrease in the corpus striatum of Huntington's disease mice","volume":"11","author":"van Dellen","year":"2000","journal-title":"NeuroReport"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0445","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","article-title":"Neuropathological classification of Huntington's disease","volume":"44","author":"Vonsattel","year":"1985","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0450","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1523\/JNEUROSCI.04-01-00084.1984","article-title":"DARPP-32, a dopamine- and adenosine 3\u2032:5\u2032-monophosphate-regulated phosphoprotein enriched in dopamine-innervated brain regions. I. Regional and cellular distribution in the rat brain","volume":"4","author":"Walaas","year":"1984","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0455","doi-asserted-by":"crossref","first-page":"3498","DOI":"10.1073\/pnas.0308679101","article-title":"Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset","volume":"101","author":"Wexler","year":"2004","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0460","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. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0465","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1093\/hmg\/11.6.633","article-title":"Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice","volume":"11","author":"Wheeler","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0470","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1093\/hmg\/11.8.905","article-title":"Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice","volume":"11","author":"Yu","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2010.12.017_bb0475","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1126\/science.1059581","article-title":"Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease","volume":"293","author":"Zuccato","year":"2001","journal-title":"Science"},{"key":"10.1016\/j.expneurol.2010.12.017_bb0480","doi-asserted-by":"crossref","first-page":"6972","DOI":"10.1523\/JNEUROSCI.4278-06.2007","article-title":"Widespread disruption of repressor element-1 silencing transcription factor\/neuron-restrictive silencer factor occupancy at its target genes in Huntington's disease","volume":"27","author":"Zuccato","year":"2007","journal-title":"J. Neurosci."}],"container-title":["Experimental Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488610004565?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488610004565?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,16]],"date-time":"2025-09-16T05:42:12Z","timestamp":1758001332000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0014488610004565"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,4]]},"references-count":96,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2011,4]]}},"alternative-id":["S0014488610004565"],"URL":"https:\/\/doi.org\/10.1016\/j.expneurol.2010.12.017","relation":{},"ISSN":["0014-4886"],"issn-type":[{"value":"0014-4886","type":"print"}],"subject":[],"published":{"date-parts":[[2011,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats","name":"articletitle","label":"Article Title"},{"value":"Experimental Neurology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.expneurol.2010.12.017","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2010 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}