{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T11:58:15Z","timestamp":1779278295665,"version":"3.51.4"},"reference-count":43,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2019,3,1]],"date-time":"2019-03-01T00:00:00Z","timestamp":1551398400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,3,1]],"date-time":"2019-03-01T00:00:00Z","timestamp":1551398400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2019,10,30]],"date-time":"2019-10-30T00:00:00Z","timestamp":1572393600000},"content-version":"am","delay-in-days":243,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100000005","name":"Department of Defense","doi-asserted-by":"crossref","award":["W81XWH-14-1-0282"],"award-info":[{"award-number":["W81XWH-14-1-0282"]}],"id":[{"id":"10.13039\/100000005","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["NS081282"],"award-info":[{"award-number":["NS081282"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"name":"International Mobility, Programme Salvador de Madariaga","award":["PRX15\/00395"],"award-info":[{"award-number":["PRX15\/00395"]}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Brain Research"],"published-print":{"date-parts":[[2019,3]]},"DOI":"10.1016\/j.brainres.2018.10.025","type":"journal-article","created":{"date-parts":[[2018,10,23]],"date-time":"2018-10-23T02:17:23Z","timestamp":1540261043000},"page":"24-31","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":13,"special_numbering":"C","title":["Efficient RNA interference-based knockdown of mutant torsinA reveals reversibility of PERK-eIF2\u03b1 pathway dysregulation in DYT1 transgenic rats in vivo"],"prefix":"10.1016","volume":"1706","author":[{"given":"Genevieve","family":"Beauvais","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jaime L.","family":"Watson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jose A.","family":"Aguirre","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luis","family":"Tecedor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michelle E.","family":"Ehrlich","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pedro","family":"Gonzalez-Alegre","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.brainres.2018.10.025_b0005","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1111\/j.1471-4159.2007.04590.x","article-title":"Dopamine release is impaired in a mouse model of DYT1 dystonia","volume":"102","author":"Balcioglu","year":"2007","journal-title":"J. Neurochem."},{"key":"10.1016\/j.brainres.2018.10.025_b0010","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1111\/ene.12650","article-title":"Isolated and combined dystonia syndromes - an update on new genes and their phenotypes","volume":"22","author":"Balint","year":"2015","journal-title":"Eur. J. Neurol."},{"key":"10.1016\/j.brainres.2018.10.025_b0015","doi-asserted-by":"crossref","first-page":"10245","DOI":"10.1523\/JNEUROSCI.0669-16.2016","article-title":"Disruption of protein processing in the endoplasmic reticulum of DYT1 knock-in mice implicates novel pathways in dystonia pathogenesis","volume":"36","author":"Beauvais","year":"2016","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainres.2018.10.025_b0020","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1016\/j.neuroscience.2017.12.033","article-title":"Exploring the interaction between eIF2alpha dysregulation, acute endoplasmic reticulum stress and DYT1 dystonia in the mammalian brain","volume":"371","author":"Beauvais","year":"2018","journal-title":"Neuroscience"},{"key":"10.1016\/j.brainres.2018.10.025_b0025","doi-asserted-by":"crossref","DOI":"10.1002\/cm.21294","article-title":"Translational control by eIF2alpha in neurons: beyond the stress response","author":"Bellato","year":"2016","journal-title":"Cytoskeleton (Hoboken)"},{"key":"10.1016\/j.brainres.2018.10.025_b0030","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0042644","article-title":"DYT1 knock-in mice are not sensitized against mitochondrial complex-II inhibition","volume":"7","author":"Bode","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.brainres.2018.10.025_b0035","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1016\/j.nbd.2010.11.015","article-title":"Molecular pathways in dystonia","volume":"42","author":"Bragg","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainres.2018.10.025_b0040","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/S0896-6273(01)00350-6","article-title":"TorsinA: movement at many levels","volume":"31","author":"Breakefield","year":"2001","journal-title":"Neuron"},{"key":"10.1016\/j.brainres.2018.10.025_b0045","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.brainres.2018.10.025_b0050","doi-asserted-by":"crossref","first-page":"1746","DOI":"10.1212\/WNL.54.9.1746","article-title":"The DYT1 phenotype and guidelines for diagnostic testing","volume":"54","author":"Bressman","year":"2000","journal-title":"Neurology"},{"key":"10.1016\/j.brainres.2018.10.025_b0055","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0067680","article-title":"Capsid serotype and timing of injection determines AAV transduction in the neonatal mice brain","volume":"8","author":"Chakrabarty","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/j.brainres.2018.10.025_b0060","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.cell.2007.01.050","article-title":"eIF2alpha phosphorylation bidirectionally regulates the switch from short- to long-term synaptic plasticity and memory","volume":"129","author":"Costa-Mattioli","year":"2007","journal-title":"Cell"},{"key":"10.1016\/j.brainres.2018.10.025_b0065","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/S1877-1173(09)90008-4","article-title":"Translational regulatory mechanisms in synaptic plasticity and memory storage","volume":"90","author":"Costa-Mattioli","year":"2009","journal-title":"Prog. Mol. Biol. Transl. Sci."},{"key":"10.1016\/j.brainres.2018.10.025_b0070","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1016\/j.expneurol.2005.08.025","article-title":"Generation and characterization of Dyt1 DeltaGAG knock-in mouse as a model for early-onset dystonia","volume":"196","author":"Dang","year":"2005","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.brainres.2018.10.025_b0075","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1007\/978-3-7091-0932-8_18","article-title":"Integrating neurotransmission in striatal medium spiny neurons","volume":"970","author":"Girault","year":"2012","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1016\/j.brainres.2018.10.025_b0080","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/B978-0-12-396456-4.00006-7","article-title":"Signaling in striatal neurons: the phosphoproteins of reward, addiction, and dyskinesia","volume":"106","author":"Girault","year":"2012","journal-title":"Prog. Mol. Biol. Transl. Sci."},{"key":"10.1016\/j.brainres.2018.10.025_b0085","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1002\/ana.10548","article-title":"Toward therapy for DYT1 dystonia: allele-specific silencing of mutant TorsinA","volume":"53","author":"Gonzalez-Alegre","year":"2003","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.brainres.2018.10.025_b0090","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.brainres.2018.10.025_b0095","doi-asserted-by":"crossref","first-page":"10502","DOI":"10.1523\/JNEUROSCI.3016-05.2005","article-title":"Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1 dystonia","volume":"25","author":"Gonzalez-Alegre","year":"2005","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainres.2018.10.025_b0100","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.brainres.2018.10.025_b0105","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.brainres.2018.10.025_b0110","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/j.devcel.2016.06.017","article-title":"Torsins are essential regulators of cellular lipid metabolism","volume":"38","author":"Grillet","year":"2016","journal-title":"Dev. Cell."},{"key":"10.1016\/j.brainres.2018.10.025_b0115","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.nbd.2012.03.024","article-title":"Generation of a novel rodent model for DYT1 dystonia","volume":"47","author":"Grundmann","year":"2012","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainres.2018.10.025_b0120","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":"Proceed. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.brainres.2018.10.025_b0125","doi-asserted-by":"crossref","first-page":"1436","DOI":"10.1093\/hmg\/ddn032","article-title":"siRNA knock-down of mutant torsinA restores processing through secretory pathway in DYT1 dystonia cells","volume":"17","author":"Hewett","year":"2008","journal-title":"Human Mol. Genet."},{"key":"10.1016\/j.brainres.2018.10.025_b0130","doi-asserted-by":"crossref","first-page":"3080","DOI":"10.1172\/JCI72830","article-title":"TorsinA hypofunction causes abnormal twisting movements and sensorimotor circuit neurodegeneration","volume":"124","author":"Liang","year":"2014","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.brainres.2018.10.025_b0135","doi-asserted-by":"crossref","first-page":"2336","DOI":"10.1093\/brain\/awp194","article-title":"Impairment of bidirectional synaptic plasticity in the striatum of a mouse model of DYT1 dystonia: role of endogenous acetylcholine","volume":"132","author":"Martella","year":"2009","journal-title":"Brain"},{"key":"10.1016\/j.brainres.2018.10.025_b0140","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1038\/gt.2011.10","article-title":"Lethal toxicity caused by expression of shRNA in the mouse striatum: implications for therapeutic design","volume":"18","author":"Martin","year":"2011","journal-title":"Gene Ther."},{"key":"10.1016\/j.brainres.2018.10.025_b0145","doi-asserted-by":"crossref","first-page":"5868","DOI":"10.1073\/pnas.0801775105","article-title":"Artificial miRNAs mitigate shRNA-mediated toxicity in the brain: implications for the therapeutic development of RNAi","volume":"105","author":"McBride","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.brainres.2018.10.025_b0150","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.brainres.2018.10.025_b0155","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.brainres.2018.10.025_b0160","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/j.nbd.2010.04.016","article-title":"Cell-autonomous alteration of dopaminergic transmission by wild type and mutant (DeltaE) TorsinA in transgenic mice","volume":"39","author":"Page","year":"2010","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainres.2018.10.025_b0165","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.17111","article-title":"Impairment of cocaine-mediated behaviours in mice by clinically relevant Ras-ERK inhibitors","volume":"5","author":"Papale","year":"2016","journal-title":"Elife"},{"key":"10.1016\/j.brainres.2018.10.025_b0170","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.08352","article-title":"Forebrain deletion of the dystonia protein torsinA causes dystonic-like movements and loss of striatal cholinergic neurons","volume":"4","author":"Pappas","year":"2015","journal-title":"Elife"},{"key":"10.1016\/j.brainres.2018.10.025_b0175","doi-asserted-by":"crossref","DOI":"10.1007\/s00702-018-1864-6","article-title":"Synaptic plasticity and levodopa-induced dyskinesia: electrophysiological and structural abnormalities","author":"Picconi","year":"2018","journal-title":"J. Neu. Transm. (Vienna)"},{"key":"10.1016\/j.brainres.2018.10.025_b0180","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1016\/j.neuron.2016.11.012","article-title":"Functional genomic analyses of mendelian and sporadic disease identify impaired eIF2alpha signaling as a generalizable mechanism for dystonia","volume":"92","author":"Rittiner","year":"2016","journal-title":"Neuron"},{"key":"10.1016\/j.brainres.2018.10.025_b0185","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.nbd.2012.05.009","article-title":"Functional analysis of dopaminergic systems in a DYT1 knock-in mouse model of dystonia","volume":"48","author":"Song","year":"2012","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainres.2018.10.025_b0190","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1038\/nrneurol.2009.160","article-title":"Primary dystonia: molecules and mechanisms","volume":"5","author":"Tanabe","year":"2009","journal-title":"Nat. Rev. Neurol."},{"key":"10.1016\/j.brainres.2018.10.025_b0195","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 DeltaE-torsinA mutant","volume":"101","author":"Torres","year":"2004","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.brainres.2018.10.025_b0200","doi-asserted-by":"crossref","first-page":"6459","DOI":"10.1093\/hmg\/ddv355","article-title":"A novel conditional knock-in approach defines molecular and circuit effects of the DYT1 dystonia mutation","volume":"24","author":"Weisheit","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainres.2018.10.025_b0205","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/j.expneurol.2008.04.020","article-title":"Commentary: dopaminergic dysfunction in DYT1 dystonia","volume":"212","author":"Wichmann","year":"2008","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.brainres.2018.10.025_b0210","doi-asserted-by":"crossref","DOI":"10.1242\/bio.032839","article-title":"Dynamic nuclear envelope phenotype in rats overexpressing mutated human torsinA protein","author":"Yu-Taeger","year":"2018","journal-title":"Biol. Open"},{"key":"10.1016\/j.brainres.2018.10.025_b0215","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1007169","article-title":"Mutations in THAP1\/DYT6 reveal that diverse dystonia genes disrupt similar neuronal pathways and functions","volume":"14","author":"Zakirova","year":"2018","journal-title":"PLoS Genet."}],"container-title":["Brain Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006899318305377?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006899318305377?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,13]],"date-time":"2025-09-13T17:58:48Z","timestamp":1757786328000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0006899318305377"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,3]]},"references-count":43,"alternative-id":["S0006899318305377"],"URL":"https:\/\/doi.org\/10.1016\/j.brainres.2018.10.025","relation":{},"ISSN":["0006-8993"],"issn-type":[{"value":"0006-8993","type":"print"}],"subject":[],"published":{"date-parts":[[2019,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Efficient RNA interference-based knockdown of mutant torsinA reveals reversibility of PERK-eIF2\u03b1 pathway dysregulation in DYT1 transgenic rats in vivo","name":"articletitle","label":"Article Title"},{"value":"Brain Research","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.brainres.2018.10.025","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}