{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T22:04:21Z","timestamp":1790978661714,"version":"4.1.0"},"reference-count":46,"publisher":"Oxford University Press (OUP)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,3]]},"DOI":"10.1093\/brain\/awr348","type":"journal-article","created":{"date-parts":[[2012,1,18]],"date-time":"2012-01-18T01:48:53Z","timestamp":1326851333000},"page":"833-846","source":"Crossref","is-referenced-by-count":113,"title":["Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43"],"prefix":"10.1093","volume":"135","author":[{"given":"Azusa","family":"Uchida","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroki","family":"Sasaguri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuyuki","family":"Kimura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mio","family":"Tajiri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuya","family":"Ohkubo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumiko","family":"Ono","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumika","family":"Sakaue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuaki","family":"Kanai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Hirai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuhiko","family":"Sano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazumoto","family":"Shibuya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariko","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shigefumi","family":"Yokota","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayuki","family":"Kubodera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Tomori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyohei","family":"Sakaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsuhiro","family":"Enomoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukihiko","family":"Hirai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiro","family":"Kumagai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhiro","family":"Yasutomi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideki","family":"Mochizuki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Kuwabara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiki","family":"Uchihara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidehiro","family":"Mizusawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takanori","family":"Yokota","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2012,1,17]]},"reference":[{"key":"key\n\t\t\t\t20170522091031_awr348-B1","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/j.expneurol.2005.02.006","article-title":"Spinal pathways involved in the control of forelimb motor function in rats","volume":"194","author":"Anderson","year":"2005","journal-title":"Exp Neurol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B2","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.bbrc.2006.10.093","article-title":"TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis","volume":"351","author":"Arai","year":"2006","journal-title":"Biochem Biophys Res Commun"},{"key":"key\n\t\t\t\t20170522091031_awr348-B3","doi-asserted-by":"crossref","first-page":"3206","DOI":"10.1093\/hmg\/ddq230","article-title":"Neurotoxic effects of TDP-43 overexpression in C. elegans","volume":"19","author":"Ash","year":"2010","journal-title":"Hum Mol Genet"},{"key":"key\n\t\t\t\t20170522091031_awr348-B4","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1038\/emboj.2010.310","article-title":"TDP-43 regulates its mRNA levels through a negative feedback loop","volume":"30","author":"Ayala","year":"2011","journal-title":"EMBO J"},{"key":"key\n\t\t\t\t20170522091031_awr348-B5","doi-asserted-by":"crossref","first-page":"20523","DOI":"10.1073\/pnas.1006869107","article-title":"Deficits in axonal transport precede ALS symptoms in vivo","volume":"107","author":"Bilsland","year":"2010","journal-title":"Proc Natl Acad Sci USA"},{"key":"key\n\t\t\t\t20170522091031_awr348-B6","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1080\/146608200300079536","article-title":"El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis","volume":"1","author":"Brooks","year":"2000","journal-title":"Amyotroph Lateral Scler Other Motor Neuron Disord"},{"key":"key\n\t\t\t\t20170522091031_awr348-B7","doi-asserted-by":"crossref","first-page":"36337","DOI":"10.1074\/jbc.M104236200","article-title":"Characterization and functional implications of the RNA binding properties of nuclear factor TDP-43, a novel splicing regulator of CFTR exon 9","volume":"276","author":"Buratti","year":"2001","journal-title":"J Biol Chem"},{"key":"key\n\t\t\t\t20170522091031_awr348-B8","doi-asserted-by":"crossref","first-page":"867","DOI":"10.2741\/2727","article-title":"Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease","volume":"13","author":"Buratti","year":"2008","journal-title":"Front Biosci"},{"key":"key\n\t\t\t\t20170522091031_awr348-B9","doi-asserted-by":"crossref","first-page":"2268","DOI":"10.1111\/j.1742-4658.2010.07643.x","article-title":"Nuclear factor TDP-43 can affect selected microRNA levels","volume":"277","author":"Buratti","year":"2010","journal-title":"FEBS J"},{"key":"key\n\t\t\t\t20170522091031_awr348-B10","doi-asserted-by":"crossref","first-page":"227","DOI":"10.2353\/ajpath.2007.070182","article-title":"TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions","volume":"171","author":"Cairns","year":"2007","journal-title":"Am J Pathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B11","volume-title":"Human neuroanatomy","author":"Carpenter","year":"1983","edition":"8th edn"},{"key":"key\n\t\t\t\t20170522091031_awr348-B46","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1097\/00005072-199209000-00008","article-title":"Peripherin and neurofilament protein coexist in spinal spheroids of motor neuron disease","volume":"51","author":"Corbo","year":"1992","journal-title":"J Neuropathol Exp Neurol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B12","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/j.clinph.2007.09.143","article-title":"Electrodiagnostic criteria for diagnosis of ALS","volume":"119","author":"De Carvalho","year":"2008","journal-title":"Clin Neurophysiol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B13","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1002\/mus.880130612","article-title":"Amyotrophic lateral sclerosis: Macro\u2013EMG and twitch forces of single motor units","volume":"13","author":"Dengler","year":"1990","journal-title":"Muscle Nerve"},{"key":"key\n\t\t\t\t20170522091031_awr348-B14","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1111\/j.1440-1789.2009.01091.x","article-title":"Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies","volume":"30","author":"Geser","year":"2010","journal-title":"Neuropathology"},{"key":"key\n\t\t\t\t20170522091031_awr348-B15","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/j.1750-3639.2009.00284.x","article-title":"TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis","volume":"20","author":"Giordana","year":"2010","journal-title":"Brain Pathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B16","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1007\/s00401-009-0571-7","article-title":"TARDBP 3\u2032-UTR variant in autopsy-confirmed frontotemporal lobar degeneration with TDP-43 proteinopathy","volume":"11","author":"Gitcho","year":"2009","journal-title":"Acta Neuropathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B17","doi-asserted-by":"crossref","first-page":"11068","DOI":"10.1074\/jbc.C109.078527","article-title":"Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS)","volume":"285","author":"Hanson","year":"2010","journal-title":"J Biol Chem"},{"key":"key\n\t\t\t\t20170522091031_awr348-B18","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1093\/brain\/118.3.707","article-title":"Natural history of amyotrophic lateral sclerosis in a data base population. Validation of a scoring system and a model for survival prediction","volume":"118","author":"Harverkamp","year":"1995","journal-title":"Brain"},{"key":"key\n\t\t\t\t20170522091031_awr348-B19","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1002\/ana.21425","article-title":"Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis","volume":"64","author":"Hasegawa","year":"2008","journal-title":"Ann Neurol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B20","doi-asserted-by":"crossref","first-page":"8516","DOI":"10.1074\/jbc.M809462200","article-title":"Expression of TDP-43 C-terminal fragments in vitro recapitulates pathological features of TDP-43 proteinopathies","volume":"284","author":"Igaz","year":"2009","journal-title":"J Biol Chem"},{"key":"key\n\t\t\t\t20170522091031_awr348-B21","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1172\/JCI44867","article-title":"Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice","volume":"121","author":"Igaz","year":"2011","journal-title":"J Clin Invest"},{"key":"key\n\t\t\t\t20170522091031_awr348-B22","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1093\/hmg\/ddp534","article-title":"Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo","volume":"19","author":"Kabashi","year":"2010","journal-title":"Hum Mol Genet"},{"key":"key\n\t\t\t\t20170522091031_awr348-B23","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1038\/ng.132","article-title":"TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis","volume":"40","author":"Kabashi","year":"2008","journal-title":"Nat Genet"},{"key":"key\n\t\t\t\t20170522091031_awr348-B24","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1002\/mus.21936","article-title":"Onset and spreading patterns of upper and lower motor neuron symptoms in amyotrophic lateral sclerosis","volume":"43","author":"K\u00f6rner","year":"2011","journal-title":"Muscle Nerve"},{"key":"key\n\t\t\t\t20170522091031_awr348-B25","doi-asserted-by":"crossref","first-page":"3169","DOI":"10.1073\/pnas.0913602107","article-title":"A Drosophila model for TDP-43 proteinopathy","volume":"107","author":"Li","year":"2010","journal-title":"Proc Natl Acad Sci USA"},{"key":"key\n\t\t\t\t20170522091031_awr348-B26","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1007\/s00401-011-0845-8","article-title":"A harmonized classification system for FTLD-TDP pathology","volume":"122","author":"Mackenzie","year":"2011","journal-title":"Acta Neuropathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B27","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1007\/s00401-007-0240-7","article-title":"Gene expression analysis of frontotemporal lobar degeneration of the motor neuron disease type with ubiquitinated inclusions","volume":"114","author":"Mishra","year":"2007","journal-title":"Acta Neuropathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B28","volume-title":"Amyotrophic lateral sclerosis","author":"Mitsumoto","year":"1998"},{"key":"key\n\t\t\t\t20170522091031_awr348-B29","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1097\/00005072-198801000-00002","article-title":"Accumulation of phosphorylated neurofilaments in anterior horn motoneurons of amyotrophic lateral sclerosis patients","volume":"47","author":"Munoz","year":"1988","journal-title":"J Neuropathol Exp Neurol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B30","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1007\/s00401-008-0477-9","article-title":"Phosphorylation of S409\/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies","volume":"117","author":"Neumann","year":"2009","journal-title":"Acta Neuropathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B31","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1126\/science.1134108","article-title":"Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis","volume":"314","author":"Neumann","year":"2006","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522091031_awr348-B32","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/0304-3940(93)90576-7","article-title":"Bunina bodies in amyotrophic lateral sclerosis immunostained with rabbit anti-cystatin C serum","volume":"162","author":"Okamoto","year":"1993","journal-title":"Neurosci Lett"},{"key":"key\n\t\t\t\t20170522091031_awr348-B33","doi-asserted-by":"crossref","first-page":"3584","DOI":"10.1128\/JVI.69.6.3584-3596.1995","article-title":"Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs","volume":"69","author":"Ou","year":"1995","journal-title":"J Virol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B34","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1111\/j.1750-3639.2003.tb00002.x","article-title":"Neuropathology with clinical correlations of sporadic amyotrophic lateral sclerosis:102 autopsy cases examined between 1962 and 2000","volume":"13","author":"Piao","year":"2003","journal-title":"Brain Pathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B35","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1038\/nn.2779","article-title":"Long pre-mRNA deletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43","volume":"14","author":"Polymenidou","year":"2011","journal-title":"Nat Neurosci"},{"key":"key\n\t\t\t\t20170522091031_awr348-B36","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1038\/nn1653","article-title":"Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF","volume":"9","author":"Pun","year":"2006","journal-title":"Nat Neurosci"},{"key":"key\n\t\t\t\t20170522091031_awr348-B37","doi-asserted-by":"crossref","first-page":"16325","DOI":"10.1073\/pnas.1003459107","article-title":"Altered distributions of gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice","volume":"107","author":"Shan","year":"2010","journal-title":"Proc Natl Acad Sci USA"},{"key":"key\n\t\t\t\t20170522091031_awr348-B38","doi-asserted-by":"crossref","first-page":"2610","DOI":"10.1093\/brain\/awr159","article-title":"Pathological hallmarks of amyotrophic lateral sclerosis\/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments","volume":"134","author":"Swarup","year":"2011","journal-title":"Brain"},{"key":"key\n\t\t\t\t20170522091031_awr348-B39","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1007\/s00401-007-0206-9","article-title":"TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation","volume":"113","author":"Tan","year":"2007","journal-title":"Acta Neuropathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B40","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1007\/s00401-009-0621-1","article-title":"Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutation","volume":"119","author":"Tateishi","year":"2010","journal-title":"Acta Neuropathol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B41","doi-asserted-by":"crossref","first-page":"e12247","DOI":"10.1371\/journal.pone.0012247","article-title":"TDP-43-mediated neuron loss in vivo requires RNA-binding activity","volume":"5","author":"Voigt","year":"2010","journal-title":"PLoS ONE"},{"key":"key\n\t\t\t\t20170522091031_awr348-B42","doi-asserted-by":"crossref","first-page":"3858","DOI":"10.1073\/pnas.0912417107","article-title":"TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration","volume":"107","author":"Wils","year":"2010","journal-title":"Proc Natl Acad Sci USA"},{"key":"key\n\t\t\t\t20170522091031_awr348-B43","doi-asserted-by":"crossref","first-page":"10851","DOI":"10.1523\/JNEUROSCI.1630-10.2010","article-title":"Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice","volume":"30","author":"Xu","year":"2010","journal-title":"J Neurosci"},{"key":"key\n\t\t\t\t20170522091031_awr348-B44","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1002\/ana.21392","article-title":"TDP-43 mutation in familial amyotrophic lateral sclerosis","volume":"63","author":"Yokoseki","year":"2008","journal-title":"Ann Neurol"},{"key":"key\n\t\t\t\t20170522091031_awr348-B45","doi-asserted-by":"crossref","first-page":"7607","DOI":"10.1073\/pnas.0900688106","article-title":"Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity","volume":"106","author":"Zhang","year":"2009","journal-title":"Proc Natl Acad Sci USA"}],"container-title":["Brain"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/brain\/article-pdf\/135\/3\/833\/17346993\/awr348.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,6,30]],"date-time":"2020-06-30T23:43:37Z","timestamp":1593560617000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/brain\/article-lookup\/doi\/10.1093\/brain\/awr348"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,1,17]]},"references-count":46,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2012,1,17]]},"published-print":{"date-parts":[[2012,3]]}},"URL":"https:\/\/doi.org\/10.1093\/brain\/awr348","relation":{},"ISSN":["1460-2156","0006-8950"],"issn-type":[{"value":"1460-2156","type":"electronic"},{"value":"0006-8950","type":"print"}],"subject":[],"published":{"date-parts":[[2012,1,17]]}}}