{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T04:51:28Z","timestamp":1789620688752,"version":"build-2803163510"},"reference-count":73,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2012,5,1]],"date-time":"2012-05-01T00:00:00Z","timestamp":1335830400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2012,5,1]],"date-time":"2012-05-01T00:00:00Z","timestamp":1335830400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2012,5,1]],"date-time":"2012-05-01T00:00:00Z","timestamp":1335830400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2012,5,1]],"date-time":"2012-05-01T00:00:00Z","timestamp":1335830400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2012,5,1]],"date-time":"2012-05-01T00:00:00Z","timestamp":1335830400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2012,5,1]],"date-time":"2012-05-01T00:00:00Z","timestamp":1335830400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2012,5,1]],"date-time":"2012-05-01T00:00:00Z","timestamp":1335830400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Behavioural Brain Research"],"published-print":{"date-parts":[[2012,5]]},"DOI":"10.1016\/j.bbr.2012.02.029","type":"journal-article","created":{"date-parts":[[2012,2,25]],"date-time":"2012-02-25T10:34:54Z","timestamp":1330166094000},"page":"389-398","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":32,"title":["Improved motor performance in Dyt1 \u0394GAG heterozygous knock-in mice by cerebellar Purkinje-cell specific Dyt1 conditional knocking-out"],"prefix":"10.1016","volume":"230","author":[{"given":"Fumiaki","family":"Yokoi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mai Tu","family":"Dang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuqing","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbr.2012.02.029_bib0005","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1038\/ng0997-40","article-title":"The early-onset torsion dystonia gene (DYT1) encodes an ATP-binding protein","volume":"17","author":"Ozelius","year":"1997","journal-title":"Nat Genet"},{"key":"10.1016\/j.bbr.2012.02.029_bib0010","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1002\/ana.410430518","article-title":"Expression of the early-onset torsion dystonia gene (DYT1) in human brain","volume":"43","author":"Augood","year":"1998","journal-title":"Ann Neurol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0015","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1002\/1531-8249(199911)46:5<761::AID-ANA12>3.0.CO;2-Z","article-title":"Distribution of the mRNAs encoding torsinA and torsinB in the normal adult human brain","volume":"46","author":"Augood","year":"1999","journal-title":"Ann Neurol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0020","doi-asserted-by":"crossref","first-page":"15650","DOI":"10.1073\/pnas.0308088101","article-title":"Effect of torsinA on membrane proteins reveals a loss of function and a dominant-negative phenotype of the dystonia-associated \u0394E-torsinA mutant","volume":"101","author":"Torres","year":"2004","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.bbr.2012.02.029_bib0025","doi-asserted-by":"crossref","first-page":"7271","DOI":"10.1073\/pnas.0701185104","article-title":"Mutant torsinA interferes with protein processing through the secretory pathway in DYT1 dystonia cells","volume":"104","author":"Hewett","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.bbr.2012.02.029_bib0030","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1038\/ng0295-152","article-title":"Genetic analysis of idiopathic torsion dystonia in Ashkenazi Jews and their recent descent from a small founder population","volume":"9","author":"Risch","year":"1995","journal-title":"Nat Genet"},{"key":"10.1016\/j.bbr.2012.02.029_bib0035","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1002\/mds.10096","article-title":"Intrafamilial phenotypic variability of the DYT1 dystonia: from asymptomatic TOR1A gene carrier status to dystonic storm","volume":"17","author":"Opal","year":"2002","journal-title":"Mov Disord"},{"key":"10.1016\/j.bbr.2012.02.029_bib0040","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1136\/jnnp.2008.148270","article-title":"Novel TOR1A mutation p.Arg288Gln in early-onset dystonia (DYT1)","volume":"79","author":"Zirn","year":"2008","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"10.1016\/j.bbr.2012.02.029_bib0045","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1007\/s100480100111","article-title":"Novel mutation in the TOR1A (DYT1) gene in atypical early onset dystonia and polymorphisms in dystonia and early onset parkinsonism","volume":"3","author":"Leung","year":"2001","journal-title":"Neurogenetics"},{"key":"10.1016\/j.bbr.2012.02.029_bib0050","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1002\/ana.10358","article-title":"Epsilon-sarcoglycan mutations found in combination with other dystonia gene mutations","volume":"52","author":"Klein","year":"2002","journal-title":"Ann Neurol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0055","doi-asserted-by":"crossref","first-page":"1244","DOI":"10.1212\/WNL.59.8.1244","article-title":"Clinical findings of a myoclonus-dystonia family with two distinct mutations","volume":"59","author":"Doheny","year":"2002","journal-title":"Neurology"},{"key":"10.1016\/j.bbr.2012.02.029_bib0060","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1136\/jnnp.2008.162099","article-title":"Myoclonus-dystonia: clinical and genetic evaluation of a large cohort","volume":"80","author":"Ritz","year":"2009","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"10.1016\/j.bbr.2012.02.029_bib0065","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1016\/j.expneurol.2005.08.025","article-title":"Generation and characterization of Dyt1 \u0394GAG knock-in mouse as a model for early-onset dystonia","volume":"196","author":"Dang","year":"2005","journal-title":"Exp Neurol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0070","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1016\/j.neuron.2005.11.010","article-title":"Loss of the dystonia-associated protein torsinA selectively disrupts the neuronal nuclear envelope","volume":"48","author":"Goodchild","year":"2005","journal-title":"Neuron"},{"key":"10.1016\/j.bbr.2012.02.029_bib0075","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1093\/jb\/mvm191","article-title":"Motor deficits and hyperactivity in cerebral cortex-specific Dyt1 conditional knockout mice","volume":"143","author":"Yokoi","year":"2008","journal-title":"J Biochem"},{"key":"10.1016\/j.bbr.2012.02.029_bib0080","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1038\/nrn2337","article-title":"The pathophysiological basis of dystonias","volume":"9","author":"Breakefield","year":"2008","journal-title":"Nat Rev Neurosci"},{"key":"10.1016\/j.bbr.2012.02.029_bib0085","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1002\/mds.870100424","article-title":"Hemidystonia consequent upon ipsilateral vertebral artery occlusion and cerebellar infarction","volume":"10","author":"Rumbach","year":"1995","journal-title":"Mov Disord"},{"key":"10.1016\/j.bbr.2012.02.029_bib0090","doi-asserted-by":"crossref","first-page":"1769","DOI":"10.1212\/01.wnl.0000244484.60489.50","article-title":"Predominant dystonia with marked cerebellar atrophy: a rare phenotype in familial dystonia","volume":"67","author":"Le Ber","year":"2006","journal-title":"Neurology"},{"key":"10.1016\/j.bbr.2012.02.029_bib0095","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1212\/01.wnl.0000266591.49624.1a","article-title":"Structural abnormalities in the cerebellum and sensorimotor circuit in writer's cramp","volume":"69","author":"Delmaire","year":"2007","journal-title":"Neurology"},{"key":"10.1016\/j.bbr.2012.02.029_bib0100","doi-asserted-by":"crossref","first-page":"1228","DOI":"10.1212\/01.WNL.0000094240.93745.83","article-title":"Motor circuit gray matter changes in idiopathic cervical dystonia","volume":"61","author":"Draganski","year":"2003","journal-title":"Neurology"},{"key":"10.1016\/j.bbr.2012.02.029_bib0105","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1002\/mds.21495","article-title":"Morphometric changes of sensorimotor structures in focal dystonia","volume":"22","author":"Obermann","year":"2007","journal-title":"Mov Disord"},{"key":"10.1016\/j.bbr.2012.02.029_bib0110","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1093\/brain\/awm243","article-title":"Increased cerebellar activation during sequence learning in DYT1 carriers: an equiperformance study","volume":"131","author":"Carbon","year":"2008","journal-title":"Brain"},{"key":"10.1016\/j.bbr.2012.02.029_bib0115","doi-asserted-by":"crossref","first-page":"7825","DOI":"10.1523\/JNEUROSCI.22-17-07825.2002","article-title":"Abnormal cerebellar signaling induces dystonia in mice","volume":"22","author":"Pizoli","year":"2002","journal-title":"J Neurosci"},{"key":"10.1016\/j.bbr.2012.02.029_bib0120","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1038\/nn.2753","article-title":"The neural substrates of rapid-onset dystonia-parkinsonism","volume":"14","author":"Calderon","year":"2011","journal-title":"Nat Neurosci"},{"key":"10.1016\/j.bbr.2012.02.029_bib0125","doi-asserted-by":"crossref","first-page":"1925","DOI":"10.1523\/JNEUROSCI.04-08-01925.1984","article-title":"Characterization of the rat mutant dystonic (dt): a new animal model of dystonia musculorum deformans","volume":"4","author":"Lorden","year":"1984","journal-title":"J Neurosci"},{"key":"10.1016\/j.bbr.2012.02.029_bib0130","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/j.molbrainres.2005.09.009","article-title":"Caytaxin deficiency causes generalized dystonia in rats","volume":"141","author":"Xiao","year":"2005","journal-title":"Brain Res Mol Brain Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0135","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.neuroscience.2006.09.042","article-title":"Caytaxin deficiency disrupts signaling pathways in cerebellar cortex","volume":"144","author":"Xiao","year":"2007","journal-title":"Neuroscience"},{"key":"10.1016\/j.bbr.2012.02.029_bib0140","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1016\/S0092-8674(00)81381-1","article-title":"Absence epilepsy in tottering mutant mice is associated with calcium channel defects","volume":"87","author":"Fletcher","year":"1996","journal-title":"Cell"},{"key":"10.1016\/j.bbr.2012.02.029_bib0145","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1006\/exnr.1993.1064","article-title":"Cerebellectomy eliminates the motor syndrome of the genetically dystonic rat","volume":"120","author":"LeDoux","year":"1993","journal-title":"Exp Neurol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0150","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/0006-8993(95)00792-O","article-title":"Selective elimination of cerebellar output in the genetically dystonic rat","volume":"697","author":"LeDoux","year":"1995","journal-title":"Brain Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0155","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1006\/exnr.1999.7171","article-title":"Tottering mouse motor dysfunction is abolished on the Purkinje cell degeneration (pcd) mutant background","volume":"160","author":"Campbell","year":"1999","journal-title":"Exp Neurol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0160","doi-asserted-by":"crossref","first-page":"2499","DOI":"10.1093\/brain\/awn168","article-title":"The basal ganglia and cerebellum interact in the expression of dystonic movement","volume":"131","author":"Neychev","year":"2008","journal-title":"Brain"},{"key":"10.1016\/j.bbr.2012.02.029_bib0165","series-title":"Current protocols in neuroscience","article-title":"Motor coordination and balance in rodents","author":"Carter","year":"2001"},{"key":"10.1016\/j.bbr.2012.02.029_bib0170","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1093\/jb\/mvj138","article-title":"Myoclonus, motor deficits, alterations in emotional responses and monoamine metabolism in \u025b-sarcoglycan deficient mice","volume":"140","author":"Yokoi","year":"2006","journal-title":"J Biochem"},{"key":"10.1016\/j.bbr.2012.02.029_bib0175","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1016\/j.neures.2006.09.005","article-title":"Motor deficits and hyperactivity in Dyt1 knockdown mice","volume":"56","author":"Dang","year":"2006","journal-title":"Neurosci Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0180","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1093\/jb\/mvq078","article-title":"Earlier onset of motor deficits in mice with double mutations in Dyt1 and Sgce","volume":"148","author":"Yokoi","year":"2010","journal-title":"J Biochem"},{"key":"10.1016\/j.bbr.2012.02.029_bib0185","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1242\/dmm.003715","article-title":"Chemical enhancement of torsinA function in cell and animal models of torsion dystonia","volume":"3","author":"Cao","year":"2010","journal-title":"Dis Model Mech"},{"key":"10.1016\/j.bbr.2012.02.029_bib0190","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/j.bbr.2010.09.009","article-title":"Characterization of Atp1a3 mutant mice as a model of rapid-onset dystonia with parkinsonism","volume":"216","author":"DeAndrade","year":"2011","journal-title":"Behav Brain Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0195","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1016\/j.bbr.2011.10.002","article-title":"An anticholinergic reverses motor control and corticostriatal LTD deficits in Dyt1 \u0394GAG knock-in mice","volume":"226","author":"Dang","year":"2012","journal-title":"Behav Brain Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0200","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.bbr.2011.10.024","article-title":"Abnormal nuclear envelope in the cerebellar Purkinje cells and impaired motor learning in DYT11 myoclonus-dystonia mouse models","volume":"227","author":"Yokoi","year":"2012","journal-title":"Behav Brain Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0205","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/j.neuron.2004.06.028","article-title":"Mutations in the Na+\/K+ -ATPase \u03b13 gene ATP1A3 are associated with rapid-onset dystonia parkinsonism","volume":"43","author":"de Carvalho Aguiar","year":"2004","journal-title":"Neuron"},{"key":"10.1016\/j.bbr.2012.02.029_bib0210","doi-asserted-by":"crossref","first-page":"e18357","DOI":"10.1371\/journal.pone.0018357","article-title":"Altered dendritic morphology of Purkinje cells in Dyt1 \u0394GAG knock-in and Purkinje cell-specific Dyt1 conditional knockout mice","volume":"6","author":"Zhang","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.bbr.2012.02.029_bib0215","doi-asserted-by":"crossref","first-page":"5166","DOI":"10.1073\/pnas.85.14.5166","article-title":"Site-specific DNA recombination in mammalian cells by the Cre recombinase of bacteriophage P1","volume":"85","author":"Sauer","year":"1988","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.bbr.2012.02.029_bib0220","doi-asserted-by":"crossref","first-page":"5080","DOI":"10.1093\/nar\/23.24.5080","article-title":"A cre-transgenic mouse strain for the ubiquitous deletion of loxP-flanked gene segments including deletion in germ cells","volume":"23","author":"Schwenk","year":"1995","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0225","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1002\/1526-968X(200011\/12)28:3\/4<93::AID-GENE10>3.0.CO;2-W","article-title":"Cre recombinase expression in cerebellar Purkinje cells","volume":"28","author":"Barski","year":"2000","journal-title":"Genesis"},{"key":"10.1016\/j.bbr.2012.02.029_bib0230","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1016\/j.bbrc.2004.05.204","article-title":"Increased seizure susceptibility and cortical malformation in beta-catenin mutant mice","volume":"320","author":"Campos","year":"2004","journal-title":"Biochem Biophys Res Commun"},{"key":"10.1016\/j.bbr.2012.02.029_bib0235","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/j.neures.2009.07.004","article-title":"Increased c-fos expression in the central nucleus of the amygdala and enhancement of cued fear memory in Dyt1 \u0394GAG knock-in mice","volume":"65","author":"Yokoi","year":"2009","journal-title":"Neurosci Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0240","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1016\/S0306-4522(02)00205-1","article-title":"Subacute systemic 3-nitropropionic acid intoxication induces a distinct motor disorder in adult C57Bl\/6 mice: behavioural and histopathological characterisation","volume":"114","author":"Fernagut","year":"2002","journal-title":"Neuroscience"},{"key":"10.1016\/j.bbr.2012.02.029_bib0245","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1016\/0003-2697(76)90527-3","article-title":"A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding","volume":"72","author":"Bradford","year":"1976","journal-title":"Anal Biochem"},{"key":"10.1016\/j.bbr.2012.02.029_bib0250","doi-asserted-by":"crossref","first-page":"e24539","DOI":"10.1371\/journal.pone.0024539","article-title":"Motor deficits and decreased striatal dopamine receptor 2 binding activity in the striatum-specific Dyt1 conditional knockout mice","volume":"6","author":"Yokoi","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.bbr.2012.02.029_bib0255","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1016\/j.pneurobio.2006.02.006","article-title":"Cerebellar circuitry as a neuronal machine","volume":"78","author":"Ito","year":"2006","journal-title":"Prog Neurobiol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0260","doi-asserted-by":"crossref","first-page":"1491","DOI":"10.1038\/nn1544","article-title":"The cerebellum communicates with the basal ganglia","volume":"8","author":"Hoshi","year":"2005","journal-title":"Nat Neurosci"},{"key":"10.1016\/j.bbr.2012.02.029_bib0265","doi-asserted-by":"crossref","first-page":"2073","DOI":"10.1002\/mds.21070","article-title":"Neuropsychological profile of DYT1 dystonia","volume":"21","author":"Balas","year":"2006","journal-title":"Mov Disord"},{"key":"10.1016\/j.bbr.2012.02.029_bib0270","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0006-8993(01)03014-1","article-title":"Immunocytochemical characterization of torsin proteins in mouse brain","volume":"922","author":"Konakova","year":"2001","journal-title":"Brain Res"},{"key":"10.1016\/j.bbr.2012.02.029_bib0275","doi-asserted-by":"crossref","first-page":"1383","DOI":"10.1136\/jnnp.2010.207993","article-title":"Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes","volume":"81","author":"Andrews","year":"2010","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"10.1016\/j.bbr.2012.02.029_bib0280","first-page":"482","article-title":"Clinical trials in neurological disorders using AAV vectors: promises and challenges","volume":"6","author":"Mandel","year":"2004","journal-title":"Curr Opin Mol Ther"},{"key":"10.1016\/j.bbr.2012.02.029_bib0285","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1038\/nm1076","article-title":"RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia","volume":"10","author":"Xia","year":"2004","journal-title":"Nat Med"},{"key":"10.1016\/j.bbr.2012.02.029_bib0290","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 U S A"},{"key":"10.1016\/j.bbr.2012.02.029_bib0295","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1016\/j.nbd.2010.03.003","article-title":"Dopamine D2 receptor dysfunction is rescued by adenosine A2A receptor antagonism in a model of DYT1 dystonia","volume":"38","author":"Napolitano","year":"2010","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.bbr.2012.02.029_bib0300","first-page":"475","article-title":"Cerebellar control of cortico-striatal LTD","volume":"26","author":"Rossi","year":"2008","journal-title":"Restor Neurol Neurosci"},{"key":"10.1016\/j.bbr.2012.02.029_bib0305","doi-asserted-by":"crossref","first-page":"7612","DOI":"10.1073\/pnas.0308760101","article-title":"TorsinA in the nuclear envelope","volume":"101","author":"Naismith","year":"2004","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.bbr.2012.02.029_bib0310","doi-asserted-by":"crossref","first-page":"2593","DOI":"10.1523\/JNEUROSCI.4461-03.2004","article-title":"Aberrant cellular behavior of mutant torsinA implicates nuclear envelope dysfunction in DYT1 dystonia","volume":"24","author":"Gonzalez-Alegre","year":"2004","journal-title":"J Neurosci"},{"key":"10.1016\/j.bbr.2012.02.029_bib0315","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1073\/pnas.0304375101","article-title":"Mislocalization to the nuclear envelope: an effect of the dystonia-causing torsinA mutation","volume":"101","author":"Goodchild","year":"2004","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.bbr.2012.02.029_bib0320","doi-asserted-by":"crossref","first-page":"9861","DOI":"10.1073\/pnas.0912877107","article-title":"A molecular mechanism underlying the neural-specific defect in torsinA mutant mice","volume":"107","author":"Kim","year":"2010","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.bbr.2012.02.029_bib0325","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1016\/j.nbd.2007.04.015","article-title":"Overexpression of human wildtype torsinA and human \u0394GAG torsinA in a transgenic mouse model causes phenotypic abnormalities","volume":"27","author":"Grundmann","year":"2007","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.bbr.2012.02.029_bib0330","doi-asserted-by":"crossref","first-page":"2712","DOI":"10.1093\/hmg\/ddn173","article-title":"Dystonia-associated mutations cause premature degradation of torsinA protein and cell-type-specific mislocalization to the nuclear envelope","volume":"17","author":"Giles","year":"2008","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.bbr.2012.02.029_bib0335","doi-asserted-by":"crossref","first-page":"588","DOI":"10.1016\/j.neuroscience.2008.09.028","article-title":"Consequences of the DYT1 mutation on torsinA oligomerization and degradation","volume":"157","author":"Gordon","year":"2008","journal-title":"Neuroscience"},{"key":"10.1016\/j.bbr.2012.02.029_bib0340","doi-asserted-by":"crossref","first-page":"2005","DOI":"10.1093\/brain\/awp172","article-title":"The monogenic primary dystonias","volume":"132","author":"Muller","year":"2009","journal-title":"Brain"},{"key":"10.1016\/j.bbr.2012.02.029_bib0345","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1002\/ana.22138","article-title":"Direct interaction between causative genes of DYT1 and DYT6 primary dystonia","volume":"68","author":"Gavarini","year":"2010","journal-title":"Ann Neurol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0350","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1002\/ana.22157","article-title":"The dystonia gene DYT1 is repressed by the transcription factor THAP1 (DYT6)","volume":"68","author":"Kaiser","year":"2010","journal-title":"Ann Neurol"},{"key":"10.1016\/j.bbr.2012.02.029_bib0355","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1038\/ejhg.2011.159","article-title":"Identification and functional analysis of novel THAP1 mutations","volume":"20","author":"Lohmann","year":"2012","journal-title":"Eur J Hum Genet"},{"key":"10.1016\/j.bbr.2012.02.029_bib0360","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1093\/hmg\/ddl472","article-title":"SGCE missense mutations that cause myoclonus-dystonia syndrome impair \u025b-sarcoglycan trafficking to the plasma membrane: modulation by ubiquitination and torsinA","volume":"16","author":"Esapa","year":"2007","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.bbr.2012.02.029_bib0365","doi-asserted-by":"crossref","first-page":"916","DOI":"10.1093\/hmg\/ddr528","article-title":"Abnormal nuclear envelopes in the striatum and motor deficits in DYT11 myoclonus-dystonia mouse models","volume":"21","author":"Yokoi","year":"2012","journal-title":"Hum Mol Genet"}],"container-title":["Behavioural Brain Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0166432812001398?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0166432812001398?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,28]],"date-time":"2025-09-28T13:00:43Z","timestamp":1759064443000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0166432812001398"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,5]]},"references-count":73,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2012,5]]}},"alternative-id":["S0166432812001398"],"URL":"https:\/\/doi.org\/10.1016\/j.bbr.2012.02.029","relation":{},"ISSN":["0166-4328"],"issn-type":[{"value":"0166-4328","type":"print"}],"subject":[],"published":{"date-parts":[[2012,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Improved motor performance in Dyt1 \u0394GAG heterozygous knock-in mice by cerebellar Purkinje-cell specific Dyt1 conditional knocking-out","name":"articletitle","label":"Article Title"},{"value":"Behavioural Brain Research","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bbr.2012.02.029","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2012 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}