{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T22:00:54Z","timestamp":1792706454251,"version":"4.5.0"},"reference-count":59,"publisher":"Springer Science and Business Media LLC","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2013,2]]},"DOI":"10.1007\/s00401-012-1043-z","type":"journal-article","created":{"date-parts":[[2012,9,8]],"date-time":"2012-09-08T06:39:41Z","timestamp":1347086381000},"page":"273-288","source":"Crossref","is-referenced-by-count":276,"title":["Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion"],"prefix":"10.1007","volume":"125","author":[{"given":"Jacqueline C.","family":"Mitchell","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"McGoldrick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Vance","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tibor","family":"Hortobagyi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jemeen","family":"Sreedharan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Boris","family":"Rogelj","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth L.","family":"Tudor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bradley N.","family":"Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Klasen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher C. J.","family":"Miller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Cooper","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Greensmith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher E.","family":"Shaw","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2012,9,9]]},"reference":[{"key":"1043_CR1","doi-asserted-by":"crossref","unstructured":"Aoki N, Higashi S, Kawakami I, Kobayashi Z, Hosokawa M, Katsuse O, Togo T, Hirayasu Y, Akiyama H (2012) Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain. Acta Neuropathol 124:383\u2013394","DOI":"10.1007\/s00401-012-0984-6"},{"key":"1043_CR2","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.bbrc.2006.10.093","volume":"351","author":"T Arai","year":"2006","unstructured":"Arai T, Hasegawa M, Akiyama H, Ikeda K, Nonaka T, Mori H, Mann D, Tsuchiya K, Yoshida M, Hashizume Y, Oda T (2006) TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun 351:602\u2013611","journal-title":"Biochem Biophys Res Commun"},{"key":"1043_CR3","doi-asserted-by":"crossref","first-page":"3778","DOI":"10.1242\/jcs.038950","volume":"121","author":"YM Ayala","year":"2008","unstructured":"Ayala YM, Zago P, D\u2019Ambrogio A, Xu YF, Petrucelli L, Buratti E, Baralle FE (2008) Structural determinants of the cellular localization and shuttling of TDP-43. J Cell Sci 121:3778\u20133785","journal-title":"J Cell Sci"},{"key":"1043_CR4","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1016\/j.neulet.2004.12.071","volume":"379","author":"A Belly","year":"2005","unstructured":"Belly A, Moreau-Gachelin F, Sadoul R, Goldberg Y (2005) Delocalization of the multifunctional RNA splicing factor TLS\/FUS in hippocampal neurones: exclusion from the nucleus and accumulation in dendritic granules and spine heads. Neurosci Lett 379:152\u2013157","journal-title":"Neurosci Lett"},{"key":"1043_CR5","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/S1050-3862(96)00167-2","volume":"13","author":"DR Borchelt","year":"1996","unstructured":"Borchelt DR, Davis J, Fischer M, Lee MK, Slunt HH, Ratovitsky T, Regard J, Copeland NG, Jenkins NA, Sisodia SS, Price DL (1996) A vector for expressing foreign genes in the brains and hearts of transgenic mice. Genet Anal 13:159\u2013163","journal-title":"Genet Anal"},{"key":"1043_CR6","doi-asserted-by":"crossref","first-page":"4160","DOI":"10.1093\/hmg\/ddq335","volume":"19","author":"DA Bosco","year":"2010","unstructured":"Bosco DA, Lemay N, Ko HK, Zhou H, Burke C, Kwiatkowski TJ Jr, Sapp P, McKenna-Yasek D, Brown RH Jr, Hayward LJ (2010) Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules. Hum Mol Genet 19:4160\u20134175","journal-title":"Hum Mol Genet"},{"key":"1043_CR7","doi-asserted-by":"crossref","first-page":"914","DOI":"10.1158\/0008-5472.CAN-10-0874","volume":"71","author":"GN Brooke","year":"2010","unstructured":"Brooke GN, Culley RL, Dart DA, Mann DJ, Gaughan L, McCracken SR, Robson CN, Spencer-Dene B, Gamble SC, Powell SM, Wait R, Waxman J, Walker MM, Bevan CL (2010) FUS\/TLS is a novel mediator of androgen-dependent cell-cycle progression and prostate cancer growth. Cancer Res 71:914\u2013924","journal-title":"Cancer Res"},{"key":"1043_CR8","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1007\/s13238-011-1065-7","volume":"2","author":"Y Chen","year":"2011","unstructured":"Chen Y, Yang M, Deng J, Chen X, Ye Y, Zhu L, Liu J, Ye H, Shen Y, Li Y, Rao EJ, Fushimi K, Zhou X, Bigio EH, Mesulam M, Xu Q, Wu JY (2011) Expression of human FUS protein in Drosophila leads to progressive neurodegeneration. Protein Cell 2:477\u2013486","journal-title":"Protein Cell"},{"key":"1043_CR9","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1038\/35097565","volume":"2","author":"DW Cleveland","year":"2001","unstructured":"Cleveland DW, Rothstein JD (2001) From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat Rev Neurosci 2:806\u2013819","journal-title":"Nat Rev Neurosci"},{"key":"1043_CR10","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/nature10353","volume":"477","author":"HX Deng","year":"2011","unstructured":"Deng HX, Chen W, Hong ST, Boycott KM, Gorrie GH, Siddique N, Yang Y, Fecto F, Shi Y, Zhai H, Jiang H, Hirano M, Rampersaud E, Jansen GH, Donkervoort S, Bigio EH, Brooks BR, Ajroud K, Sufit RL, Haines JL, Mugnaini E, Pericak-Vance MA, Siddique T (2011) Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS\/dementia. Nature 477:211\u2013215","journal-title":"Nature"},{"key":"1043_CR11","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1001\/archneurol.2010.101","volume":"67","author":"HX Deng","year":"2010","unstructured":"Deng HX, Zhai H, Bigio EH, Yan J, Fecto F, Ajroud K, Mishra M, Ajroud-Driss S, Heller S, Sufit R, Siddique N, Mugnaini E, Siddique T (2010) FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis. Ann Neurol 67:739\u2013748","journal-title":"Ann Neurol"},{"key":"1043_CR12","doi-asserted-by":"crossref","unstructured":"Dormann D, Haass C (2011) TDP-43 and FUS: a nuclear affair. Trends Neurosci 34:339\u2013348","DOI":"10.1016\/j.tins.2011.05.002"},{"key":"1043_CR13","doi-asserted-by":"crossref","first-page":"2841","DOI":"10.1038\/emboj.2010.143","volume":"29","author":"D Dormann","year":"2010","unstructured":"Dormann D, Rodde R, Edbauer D, Bentmann E, Fischer I, Hruscha A, Than ME, Mackenzie IR, Capell A, Schmid B, Neumann M, Haass C (2010) ALS-associated fused in sarcoma (FUS) mutations disrupt transportin-mediated nuclear import. EMBO J 29:2841\u20132857","journal-title":"EMBO J"},{"key":"1043_CR14","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1016\/j.cub.2005.01.058","volume":"15","author":"R Fujii","year":"2005","unstructured":"Fujii R, Okabe S, Urushido T, Inoue K, Yoshimura A, Tachibana T, Nishikawa T, Hicks GG, Takumi T (2005) The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology. Curr Biol 15:587\u2013593","journal-title":"Curr Biol"},{"key":"1043_CR15","doi-asserted-by":"crossref","first-page":"5755","DOI":"10.1242\/jcs.02692","volume":"118","author":"R Fujii","year":"2005","unstructured":"Fujii R, Takumi T (2005) TLS facilitates transport of mRNA encoding an actin-stabilizing protein to dendritic spines. J Cell Sci 118:5755\u20135765","journal-title":"J Cell Sci"},{"key":"1043_CR16","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1007\/s13238-011-1014-5","volume":"2","author":"K Fushimi","year":"2011","unstructured":"Fushimi K, Long C, Jayaram N, Chen X, Li L, Wu JY (2011) Expression of human FUS\/TLS in yeast leads to protein aggregation and cytotoxicity, recapitulating key features of FUS proteinopathy. Protein Cell 2:141\u2013149","journal-title":"Protein Cell"},{"issue":"2323","key":"1043_CR17","first-page":"e2327","volume":"32","author":"J Gal","year":"2010","unstructured":"Gal J, Zhang J, Kwinter DM, Zhai J, Jia H, Jia J, Zhu H (2010) Nuclear localization sequence of FUS and induction of stress granules by ALS mutants. Neurobiol Aging 32(2323):e2327\u2013e2340","journal-title":"Neurobiol Aging"},{"key":"1043_CR18","doi-asserted-by":"crossref","first-page":"e17808","DOI":"10.1371\/journal.pone.0017808","volume":"6","author":"VK Godena","year":"2011","unstructured":"Godena VK, Romano G, Romano M, Appocher C, Klima R, Buratti E, Baralle FE, Feiguin F (2011) TDP-43 regulates Drosophila neuromuscular junctions growth by modulating Futsch\/MAP1B levels and synaptic microtubules organization. PLoS ONE 6:e17808","journal-title":"PLoS ONE"},{"key":"1043_CR19","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1002\/pmic.200400892","volume":"5","author":"I Guillemin","year":"2005","unstructured":"Guillemin I, Becker M, Ociepka K, Friauf E, Nothwang HG (2005) A subcellular prefractionation protocol for minute amounts of mammalian cell cultures and tissue. Proteomics 5:35\u201345","journal-title":"Proteomics"},{"key":"1043_CR20","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1038\/72842","volume":"24","author":"GG Hicks","year":"2000","unstructured":"Hicks GG, Singh N, Nashabi A, Mai S, Bozek G, Klewes L, Arapovic D, White EK, Koury MJ, Oltz EM, Van Kaer L, Ruley HE (2000) Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death. Nat Genet 24:175\u2013179","journal-title":"Nat Genet"},{"key":"1043_CR21","doi-asserted-by":"crossref","first-page":"e1002011","DOI":"10.1371\/journal.pgen.1002011","volume":"7","author":"C Huang","year":"2011","unstructured":"Huang C, Zhou H, Tong J, Chen H, Liu YJ, Wang D, Wei X, Xia XG (2011) FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration. PLoS Genet 7:e1002011","journal-title":"PLoS Genet"},{"key":"1043_CR22","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1002\/ana.22246","volume":"69","author":"D Ito","year":"2011","unstructured":"Ito D, Seki M, Tsunoda Y, Uchiyama H, Suzuki N (2011) Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS\/TLS. Ann Neurol 69:152\u2013162","journal-title":"Ann Neurol"},{"key":"1043_CR23","doi-asserted-by":"crossref","first-page":"e1001052","DOI":"10.1371\/journal.pbio.1001052","volume":"9","author":"S Ju","year":"2011","unstructured":"Ju S, Tardiff DF, Han H, Divya K, Zhong Q, Maquat LE, Bosco DA, Hayward LJ, Brown RH Jr, Lindquist S, Ringe D, Petsko GA (2011) A yeast model of FUS\/TLS-dependent cytotoxicity. PLoS Biol 9:e1001052","journal-title":"PLoS Biol"},{"key":"1043_CR24","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1111\/j.1471-4159.2008.05595.x","volume":"107","author":"B Kalmar","year":"2008","unstructured":"Kalmar B, Novoselov S, Gray A, Cheetham ME, Margulis B, Greensmith L (2008) Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1 mouse model of ALS. J Neurochem 107:339\u2013350","journal-title":"J Neurochem"},{"key":"1043_CR25","doi-asserted-by":"crossref","first-page":"34097","DOI":"10.1074\/jbc.M110.154831","volume":"285","author":"SH Kim","year":"2010","unstructured":"Kim SH, Shanware NP, Bowler MJ, Tibbetts RS (2010) Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS\/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA. J Biol Chem 285:34097\u201334105","journal-title":"J Biol Chem"},{"key":"1043_CR26","doi-asserted-by":"crossref","first-page":"2781","DOI":"10.1093\/nar\/gkq1162","volume":"39","author":"Y Kino","year":"2011","unstructured":"Kino Y, Washizu C, Aquilanti E, Okuno M, Kurosawa M, Yamada M, Doi H, Nukina N (2011) Intracellular localization and splicing regulation of FUS\/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations. Nucleic Acids Res 39:2781\u20132798","journal-title":"Nucleic Acids Res"},{"key":"1043_CR27","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1093\/emboj\/19.3.453","volume":"19","author":"M Kuroda","year":"2000","unstructured":"Kuroda M, Sok J, Webb L, Baechtold H, Urano F, Yin Y, Chung P, de Rooij DG, Akhmedov A, Ashley T, Ron D (2000) Male sterility and enhanced radiation sensitivity in TLS(-\/-) mice. EMBO J 19:453\u2013462","journal-title":"EMBO J"},{"key":"1043_CR28","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1126\/science.1166066","volume":"323","author":"TJ Kwiatkowski Jr","year":"2009","unstructured":"Kwiatkowski TJ Jr, Bosco DA, Leclerc AL, Tamrazian E, Vanderburg CR, Russ C, Davis A, Gilchrist J, Kasarskis EJ, Munsat T, Valdmanis P, Rouleau GA, Hosler BA, Cortelli P, de Jong PJ, Yoshinaga Y, Haines JL, Pericak-Vance MA, Yan J, Ticozzi N, Siddique T, McKenna-Yasek D, Sapp PC, Horvitz HR, Landers JE, Brown RH Jr (2009) Mutations in the FUS\/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science 323:1205\u20131208","journal-title":"Science"},{"key":"1043_CR29","doi-asserted-by":"crossref","first-page":"2510","DOI":"10.1093\/hmg\/ddr150","volume":"20","author":"NA Lanson Jr","year":"2011","unstructured":"Lanson NA Jr, Maltare A, King H, Smith R, Kim JH, Taylor JP, Lloyd TE, Pandey UB (2011) A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43. Hum Mol Genet 20:2510\u20132523","journal-title":"Hum Mol Genet"},{"key":"1043_CR30","doi-asserted-by":"crossref","first-page":"47025","DOI":"10.1074\/jbc.M300503200","volume":"278","author":"JH Lee","year":"2003","unstructured":"Lee JH, Lau KF, Perkinton MS, Standen CL, Shemilt SJ, Mercken L, Cooper JD, McLoughlin DM, Miller CC (2003) The neuronal adaptor protein X11alpha reduces Abeta levels in the brains of Alzheimer\u2019s APPswe Tg2576 transgenic mice. J Biol Chem 278:47025\u201347029","journal-title":"J Biol Chem"},{"key":"1043_CR31","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1016\/j.jocn.2009.03.005","volume":"16","author":"P Lillo","year":"2009","unstructured":"Lillo P, Hodges JR (2009) Frontotemporal dementia and motor neurone disease: overlapping clinic-pathological disorders. J Clin Neurosci 16:1131\u20131135","journal-title":"J Clin Neurosci"},{"key":"1043_CR32","doi-asserted-by":"crossref","first-page":"1077","DOI":"10.1212\/WNL.59.7.1077","volume":"59","author":"C Lomen-Hoerth","year":"2002","unstructured":"Lomen-Hoerth C, Anderson T, Miller B (2002) The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 59:1077\u20131079","journal-title":"Neurology"},{"key":"1043_CR33","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1007\/s00401-007-0223-8","volume":"114","author":"IR Mackenzie","year":"2007","unstructured":"Mackenzie IR (2007) The neuropathology and clinical phenotype of FTD with progranulin mutations. Acta Neuropathol 114:49\u201354","journal-title":"Acta Neuropathol"},{"key":"1043_CR34","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1007\/s00401-010-0764-0","volume":"121","author":"IR Mackenzie","year":"2011","unstructured":"Mackenzie IR, Munoz DG, Kusaka H, Yokota O, Ishihara K, Roeber S, Kretzschmar HA, Cairns NJ, Neumann M (2011) Distinct pathological subtypes of FTLD-FUS. Acta Neuropathol 121:207\u2013218","journal-title":"Acta Neuropathol"},{"key":"1043_CR35","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1111\/j.1440-1789.2009.01029.x","volume":"29","author":"S Maekawa","year":"2009","unstructured":"Maekawa S, Leigh PN, King A, Jones E, Steele JC, Bodi I, Shaw CE, Hortobagyi T, Al-Sarraj S (2009) TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations. Neuropathology 29:672\u2013683","journal-title":"Neuropathology"},{"key":"1043_CR36","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1093\/hmg\/ddr021","volume":"20","author":"KK McDonald","year":"2011","unstructured":"McDonald KK, Aulas A, Destroismaisons L, Pickles S, Beleac E, Camu W, Rouleau GA, Vande Velde C (2011) TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1. Hum Mol Genet 20:1400\u20131410","journal-title":"Hum Mol Genet"},{"key":"1043_CR37","doi-asserted-by":"crossref","unstructured":"Miguel L, Avequin T, Delarue M, Feuillette S, Frebourg T, Campion D, Lecourtois M (2012) Accumulation of insoluble forms of FUS protein correlates with toxicity in Drosophila. Neurobiol Aging 33:1008.e1\u22121008.e15","DOI":"10.1016\/j.neurobiolaging.2011.10.008"},{"key":"1043_CR38","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1007\/s00401-009-0598-9","volume":"118","author":"DG Munoz","year":"2009","unstructured":"Munoz DG, Neumann M, Kusaka H, Yokota O, Ishihara K, Terada S, Kuroda S, Mackenzie IR (2009) FUS pathology in basophilic inclusion body disease. Acta Neuropathol 118:617\u2013627","journal-title":"Acta Neuropathol"},{"key":"1043_CR39","doi-asserted-by":"crossref","unstructured":"Murakami T, Yang SP, Xie L, Kawano T, Fu D, Mukai A, Bohm C, Chen F, Robertson J, Suzuki H, Tartaglia GG, Vendruscolo M, Kaminski Schierle GS, Chan FT, Moloney A, Crowther D, Kaminski CF, Zhen M, St George-Hyslop P (2012) ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism. Hum Mol Genet 21:1\u22129","DOI":"10.1093\/hmg\/ddr417"},{"key":"1043_CR40","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1001\/archneur.64.4.530","volume":"64","author":"JM Murphy","year":"2007","unstructured":"Murphy JM, Henry RG, Langmore S, Kramer JH, Miller BL, Lomen-Hoerth C (2007) Continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol 64:530\u2013534","journal-title":"Arch Neurol"},{"key":"1043_CR41","doi-asserted-by":"crossref","first-page":"2595","DOI":"10.1093\/brain\/awr201","volume":"134","author":"M Neumann","year":"2011","unstructured":"Neumann M, Bentmann E, Dormann D, Jawaid A, DeJesus-Hernandez M, Ansorge O, Roeber S, Kretzschmar HA, Munoz DG, Kusaka H, Yokota O, Ang LC, Bilbao J, Rademakers R, Haass C, Mackenzie IR (2011) FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations. Brain 134:2595\u20132609","journal-title":"Brain"},{"key":"1043_CR42","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1097\/nen.0b013e31803020b9","volume":"66","author":"M Neumann","year":"2007","unstructured":"Neumann M, Mackenzie IR, Cairns NJ, Boyer PJ, Markesbery WR, Smith CD, Taylor JP, Kretzschmar HA, Kimonis VE, Forman MS (2007) TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations. J Neuropathol Exp Neurol 66:152\u2013157","journal-title":"J Neuropathol Exp Neurol"},{"key":"1043_CR43","doi-asserted-by":"crossref","first-page":"2922","DOI":"10.1093\/brain\/awp214","volume":"132","author":"M Neumann","year":"2009","unstructured":"Neumann M, Rademakers R, Roeber S, Baker M, Kretzschmar HA, Mackenzie IR (2009) A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain 132:2922\u20132931","journal-title":"Brain"},{"key":"1043_CR44","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1007\/s00401-009-0581-5","volume":"118","author":"M Neumann","year":"2009","unstructured":"Neumann M, Roeber S, Kretzschmar HA, Rademakers R, Baker M, Mackenzie IR (2009) Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol 118:605\u2013616","journal-title":"Acta Neuropathol"},{"key":"1043_CR45","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1126\/science.1134108","volume":"314","author":"M Neumann","year":"2006","unstructured":"Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, Chou TT, Bruce J, Schuck T, Grossman M, Clark CM, McCluskey LF, Miller BL, Masliah E, Mackenzie IR, Feldman H, Feiden W, Kretzschmar HA, Trojanowski JQ, Lee VM (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130\u2013133","journal-title":"Science"},{"key":"1043_CR46","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1038\/nrn1971","volume":"7","author":"P Pasinelli","year":"2006","unstructured":"Pasinelli P, Brown RH (2006) Molecular biology of amyotrophic lateral sclerosis: insights from genetics. Nat Rev Neurosci 7:710\u2013723","journal-title":"Nat Rev Neurosci"},{"key":"1043_CR47","doi-asserted-by":"crossref","first-page":"586","DOI":"10.1212\/01.wnl.0000172911.39167.b6","volume":"65","author":"GM Ringholz","year":"2005","unstructured":"Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE (2005) Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology 65:586\u2013590","journal-title":"Neurology"},{"key":"1043_CR48","doi-asserted-by":"crossref","unstructured":"Seelaar H, Klijnsma KY, de Koning I, van der Lugt A, Chiu WZ, Azmani A, Rozemuller AJ, van Swieten JC (2010) Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration. J Neurol 257:747\u2212753","DOI":"10.1007\/s00415-009-5404-z"},{"key":"1043_CR49","doi-asserted-by":"crossref","first-page":"16325","DOI":"10.1073\/pnas.1003459107","volume":"107","author":"X Shan","year":"2010","unstructured":"Shan X, Chiang PM, Price DL, Wong PC (2010) Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice. Proc Natl Acad Sci USA 107:16325\u201316330","journal-title":"Proc Natl Acad Sci USA"},{"key":"1043_CR50","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1126\/science.1154584","volume":"319","author":"J Sreedharan","year":"2008","unstructured":"Sreedharan J, Blair IP, Tripathi VB, Hu X, Vance C, Rogelj B, Ackerley S, Durnall JC, Williams KL, Buratti E, Baralle F, de Belleroche J, Mitchell JD, Leigh PN, Al-Chalabi A, Miller CC, Nicholson G, Shaw CE (2008) TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science 319:1668\u20131672","journal-title":"Science"},{"key":"1043_CR51","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1038\/nn.2778","volume":"14","author":"JR Tollervey","year":"2011","unstructured":"Tollervey JR, Curk T, Rogelj B, Briese M, Cereda M, Kayikci M, Konig J, Hortobagyi T, Nishimura AL, Zupunski V, Patani R, Chandran S, Rot G, Zupan B, Shaw CE, Ule J (2011) Characterizing the RNA targets and position-dependent splicing regulation by TDP-43. Nat Neurosci 14:452\u2013458","journal-title":"Nat Neurosci"},{"key":"1043_CR52","doi-asserted-by":"crossref","first-page":"1123","DOI":"10.1093\/jnen\/60.12.1123","volume":"60","author":"JQ Trojanowski","year":"2001","unstructured":"Trojanowski JQ, Dickson D (2001) Update on the neuropathological diagnosis of frontotemporal dementias. J Neuropathol Exp Neurol 60:1123\u20131126","journal-title":"J Neuropathol Exp Neurol"},{"key":"1043_CR53","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/s00401-010-0698-6","volume":"120","author":"H Urwin","year":"2010","unstructured":"Urwin H, Josephs KA, Rohrer JD, Mackenzie IR, Neumann M, Authier A, Seelaar H, Van Swieten JC, Brown JM, Johannsen P, Nielsen JE, Holm IE, Dickson DW, Rademakers R, Graff-Radford NR, Parisi JE, Petersen RC, Hatanpaa KJ, White CL 3rd, Weiner MF, Geser F, Van Deerlin VM, Trojanowski JQ, Miller BL, Seeley WW, van der Zee J, Kumar-Singh S, Engelborghs S, De Deyn PP, Van Broeckhoven C, Bigio EH, Deng HX, Halliday GM, Kril JJ, Munoz DG, Mann DM, Pickering-Brown SM, Doodeman V, Adamson G, Ghazi-Noori S, Fisher EM, Holton JL, Revesz T, Rossor MN, Collinge J, Mead S, Isaacs AM (2010) FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol 120:33\u201341","journal-title":"Acta Neuropathol"},{"key":"1043_CR54","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1212\/WNL.0b013e3181ccc732","volume":"74","author":"T Langenhove Van","year":"2010","unstructured":"Van Langenhove T, van der Zee J, Sleegers K, Engelborghs S, Vandenberghe R, Gijselinck I, Van den Broeck M, Mattheijssens M, Peeters K, De Deyn PP, Cruts M, Van Broeckhoven C (2010) Genetic contribution of FUS to frontotemporal lobar degeneration. Neurology 74:366\u2013371","journal-title":"Neurology"},{"key":"1043_CR55","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1126\/science.1165942","volume":"323","author":"C Vance","year":"2009","unstructured":"Vance C, Rogelj B, Hortobagyi T, De Vos KJ, Nishimura AL, Sreedharan J, Hu X, Smith B, Ruddy D, Wright P, Ganesalingam J, Williams KL, Tripathi V, Al-Saraj S, Al-Chalabi A, Leigh PN, Blair IP, Nicholson G, de Belleroche J, Gallo JM, Miller CC, Shaw CE (2009) Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science 323:1208\u20131211","journal-title":"Science"},{"key":"1043_CR56","doi-asserted-by":"crossref","first-page":"18809","DOI":"10.1073\/pnas.0908767106","volume":"106","author":"I Wegorzewska","year":"2009","unstructured":"Wegorzewska I, Bell S, Cairns NJ, Miller TM, Baloh RH (2009) TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci USA 106:18809\u201318814","journal-title":"Proc Natl Acad Sci USA"},{"key":"1043_CR57","doi-asserted-by":"crossref","first-page":"10851","DOI":"10.1523\/JNEUROSCI.1630-10.2010","volume":"30","author":"YF Xu","year":"2010","unstructured":"Xu YF, Gendron TF, Zhang YJ, Lin WL, D\u2019Alton S, Sheng H, Casey MC, Tong J, Knight J, Yu X, Rademakers R, Boylan K, Hutton M, McGowan E, Dickson DW, Lewis J, Petrucelli L (2010) Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. J Neurosci 30:10851\u201310859","journal-title":"J Neurosci"},{"key":"1043_CR58","doi-asserted-by":"crossref","first-page":"2345","DOI":"10.1016\/j.cub.2006.10.024","volume":"16","author":"A Yoshimura","year":"2006","unstructured":"Yoshimura A, Fujii R, Watanabe Y, Okabe S, Fukui K, Takumi T (2006) Myosin-Va facilitates the accumulation of mRNA\/protein complex in dendritic spines. Curr Biol 16:2345\u20132351","journal-title":"Curr Biol"},{"key":"1043_CR59","doi-asserted-by":"crossref","first-page":"e1000887","DOI":"10.1371\/journal.pgen.1000887","volume":"6","author":"H Zhou","year":"2010","unstructured":"Zhou H, Huang C, Chen H, Wang D, Landel CP, Xia PY, Bowser R, Liu YJ, Xia XG (2010) Transgenic rat model of neurodegeneration caused by mutation in the TDP gene. PLoS Genet 6:e1000887","journal-title":"PLoS Genet"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.springerlink.com\/index\/pdf\/10.1007\/s00401-012-1043-z","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,7,3]],"date-time":"2019-07-03T12:20:48Z","timestamp":1562156448000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00401-012-1043-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,9,9]]},"references-count":59,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2013,2]]}},"alternative-id":["1043"],"URL":"https:\/\/doi.org\/10.1007\/s00401-012-1043-z","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,9,9]]}}}