{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T02:41:06Z","timestamp":1791513666938,"version":"4.3.4"},"reference-count":53,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2011,3,20]],"date-time":"2011-03-20T00:00:00Z","timestamp":1300579200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2011,7]]},"DOI":"10.1007\/s00401-011-0816-0","type":"journal-article","created":{"date-parts":[[2011,3,19]],"date-time":"2011-03-19T00:13:50Z","timestamp":1300493630000},"page":"99-110","source":"Crossref","is-referenced-by-count":118,"title":["The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene"],"prefix":"10.1007","volume":"122","author":[{"given":"Julie S.","family":"Snowden","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Quan","family":"Hu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Rollinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Halliwell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Robinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yvonne S.","family":"Davidson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Parastoo","family":"Momeni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atik","family":"Baborie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy D.","family":"Griffiths","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelyn","family":"Jaros","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert H.","family":"Perry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Richardson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart M.","family":"Pickering-Brown","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Neary","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M. A.","family":"Mann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2011,3,20]]},"reference":[{"key":"816_CR1","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1155\/2006\/260734","volume":"17","author":"S Amici","year":"2006","unstructured":"Amici S, Gorno-Tempini M-L, Ogar JM, Dronkers NF, Miller BL (2006) An overview of primary progressive aphasia and its variants. Behav Neurol 17:77\u201387","journal-title":"Behav Neurol"},{"key":"816_CR2","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1007\/s00401-010-0765-z","volume":"121","author":"A Baborie","year":"2011","unstructured":"Baborie A, Griffiths TD, Jaros E, Richardson A, Ferrari R, Moreno J, Momeni P, McKeith IG, Burn DJ, Duplessis D, Pal P, Rollinson S, Pickering-Brown SM, Thompson JC, Neary D, Snowden JS, Perry R, Mann DMA (2011) Pathological correlates of frontotemporal lobar degeneration in the elderly. Acta Neuropathol 121:365\u2013371","journal-title":"Acta Neuropathol"},{"key":"816_CR3","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1093\/bioinformatics\/bth457","volume":"21","author":"JC Barrett","year":"2005","unstructured":"Barrett JC, Fry B, Maller J, Daly MJ (2005) Haploview: analysis and visualization of LD and haplotype maps. Bioinformatics 21:263\u2013265","journal-title":"Bioinformatics"},{"key":"816_CR4","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1034\/j.1600-0404.2001.2000236.x","volume":"103","author":"D Bathgate","year":"2001","unstructured":"Bathgate D, Snowden JS, Varma A, Blackshaw A, Neary D (2001) Behaviour in frontotemporal dementia: a comparison with Alzheimer\u2019s disease and vascular dementia. Acta Neurol Scand 103:367\u2013373","journal-title":"Acta Neurol Scand"},{"key":"816_CR5","doi-asserted-by":"crossref","first-page":"1376","DOI":"10.1212\/01.WNL.0000139809.16817.DD","volume":"63","author":"NJ Cairns","year":"2004","unstructured":"Cairns NJ, Grossman M, Arnold SE, Burn DJ, Jaros E, Perry RH, Duyckaerts C, Stankoff B, Pillon B, Skullerud K, Cruz-Sanchez FF, Bigio EH, Mackenzie IR, Gearing M, Juncos JL, Glass JD, Yokoo H, Nakazato Y, Mosaheb S, Thorpe JR, Uryu K, Lee VM, Trojanowski JQ (2004) Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease. Neurology 63:1376\u20131384","journal-title":"Neurology"},{"key":"816_CR6","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1007\/s00401-007-0237-2","volume":"114","author":"NJ Cairns","year":"2007","unstructured":"Cairns NJ, Bigio EH, Mackenzie IRA, Neumann M, Lee VMY, Hatanpaa KJ, White CL III, Schneider JA, Tenenholz Grinberg L, Halliday G, Duyckaerts C, Lowe JS, Holm IE, Tolnay M, Okamoto K, Yokoo H, Murayama S, Woulfe J, Munoz DG, Dickson DW, Ince PG, Trojanowski JQ, Mann DMA (2007) Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol 114:2\u201322","journal-title":"Acta Neuropathol"},{"key":"816_CR7","doi-asserted-by":"crossref","first-page":"1196","DOI":"10.1016\/S0028-3932(01)00224-X","volume":"40","author":"J Crawford","year":"2002","unstructured":"Crawford J, Garthwaite PH (2002) Investigation of the single case in neuropsychology: confidence limits on the abnormality of test scores and test score differences. Neuropsychologia 40:1196\u20131208","journal-title":"Neuropsychologia"},{"key":"816_CR8","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1076\/clin.12.4.482.7241","volume":"12","author":"J Crawford","year":"1998","unstructured":"Crawford J, Howell DC (1998) Comparing an individual\u2019s test score against norms derived from small samples. Clin Neuropsychol 12:482\u2013486","journal-title":"Clin Neuropsychol"},{"key":"816_CR9","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1007\/s00401-006-0189-y","volume":"113","author":"Y Davidson","year":"2007","unstructured":"Davidson Y, Kelley T, Mackenzie IR, Pickering-Brown SM, Du Plessis D, Neary D, Snowden JS, Mann DMA (2007) Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43. Acta Neuropathol 113:521\u2013533","journal-title":"Acta Neuropathol"},{"key":"816_CR10","doi-asserted-by":"crossref","first-page":"1217","DOI":"10.1038\/ng1669","volume":"37","author":"PI Bakker de","year":"2005","unstructured":"de Bakker PI, Yelensky R, Pe\u2019er I, Gabriel SB, Daly MJ, Altshuler D (2005) Efficiency and power in genetic association studies. Nat Genet 37:1217\u20131223","journal-title":"Nat Genet"},{"key":"816_CR11","doi-asserted-by":"crossref","first-page":"2225","DOI":"10.1126\/science.1069424","volume":"296","author":"SB Gabriel","year":"2002","unstructured":"Gabriel SB, Schaffner SF, Nguyen H, Moore JM, Roy J, Blumenstiel B (2002) The structure of haplotype blocks in the human genome. Science 296:2225\u20132229","journal-title":"Science"},{"key":"816_CR12","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1002\/ana.10825","volume":"55","author":"ML Gorno-Tempini","year":"2004","unstructured":"Gorno-Tempini ML, Dronkers NF, Rankin KP, Ogar JM, Phengrasamy L, Rosen HJ, Johnson JK, Weiner MW, Miller B (2004) Cognition and anatomy of three variants of primary progressive aphasia. Ann Neurol 55:335\u2013346","journal-title":"Ann Neurol"},{"key":"816_CR13","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1212\/01.wnl.0000191307.69661.c3","volume":"66","author":"KA Josephs","year":"2006","unstructured":"Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, Duffy JR, Parisi JE, Dickson DW (2006) Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 66:41\u201348","journal-title":"Neurology"},{"key":"816_CR14","doi-asserted-by":"crossref","first-page":"1385","DOI":"10.1093\/brain\/awl078","volume":"129","author":"KA Josephs","year":"2006","unstructured":"Josephs KA, Duffy JR, Strand EA, Whitwell JL, Layton KF, Parisi JE, Hauser MF, Witte RJ, Boeve BF, Knopman DS, Dickson DW, Jack CR Jr, Petersen RC (2006) Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 129:1385\u20131398","journal-title":"Brain"},{"key":"816_CR15","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s00401-009-0547-7","volume":"118","author":"KA Josephs","year":"2009","unstructured":"Josephs KA, Stroh A, Dugger B, Dickson DW (2009) Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes. Acta Neuropathol 118:349\u2013358","journal-title":"Acta Neuropathol"},{"key":"816_CR16","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1111\/j.1468-1331.2010.02975.x","volume":"17","author":"KA Josephs","year":"2010","unstructured":"Josephs KA, Whitwell JL, Parisi JE, Petersen RC, Boeve BF, Jack CR, Dickson DW (2010) Caudate atrophy on MRI is a characteristic feature of FTLD-FUS. Eur J Neurol 17:969\u2013975","journal-title":"Eur J Neurol"},{"key":"816_CR17","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.neurobiolaging.2007.08.022","volume":"30","author":"BJ Kelley","year":"2009","unstructured":"Kelley BJ, Haidar W, Boeve BF, Baker M, Graff-Radford NR, Krefft T, Frank AR, Jack CR Jr, Shiung M, Knopman DS, Josephs KA, Parashos SA, Rademakers R, Hutton M, Pickering-Brown S, Adamson J, Kuntz KM, Dickson DW, Parisi JE, Smith GE, Ivnik RJ, Petersen RC (2009) Prominent phenotypic variability associated with mutations in progranulin. Neurobiol Aging 30:739\u2013751","journal-title":"Neurobiol Aging"},{"key":"816_CR18","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1126\/science.1166066","volume":"323","author":"TJ Kwiatkowski","year":"2009","unstructured":"Kwiatkowski TJ, 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 PA, Yoshinaga Y, Haines JL, Pericak-Vance MA, Yan J, Ticozzi N, Siddique T, Kenna-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":"816_CR19","doi-asserted-by":"crossref","first-page":"e158","DOI":"10.1093\/brain\/awq186","volume":"133","author":"CT Loy","year":"2010","unstructured":"Loy CT, McCusker E, Kril JJ, Kwok JB, Brooks WS, McCann H, Isaacs A, Halliday GM (2010) Very early-onset frontotemporal dementia with no family history predicts underlying fused in sarcoma pathology. Brain 133:e158","journal-title":"Brain"},{"key":"816_CR20","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1007\/s00401-007-0223-8","volume":"114","author":"IRA Mackenzie","year":"2007","unstructured":"Mackenzie IRA (2007) The neuropathology and clinical phenotype of FTD with progranulin mutations. Acta Neuropathol 114:49\u201354","journal-title":"Acta Neuropathol"},{"key":"816_CR21","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1007\/s00401-006-0138-9","volume":"112","author":"IRA Mackenzie","year":"2006","unstructured":"Mackenzie IRA, Baborie A, Pickering-Brown S, Du Plessis D, Jaros E, Perry RH, Neary D, Snowden JS, Mann DMA (2006) Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype. Acta Neuropathol 112:539\u2013549","journal-title":"Acta Neuropathol"},{"key":"816_CR22","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1093\/brain\/awn061","volume":"131","author":"IRA Mackenzie","year":"2008","unstructured":"Mackenzie IRA, Foti D, Woulfe J, Hurwitz TA (2008) Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions. Brain 131:1282\u20131293","journal-title":"Brain"},{"key":"816_CR23","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1007\/s00401-008-0460-5","volume":"117","author":"IRA Mackenzie","year":"2009","unstructured":"Mackenzie IRA, Neumann M, Bigio EH, Cairns NJ, Alafuzoff I, Kril J, Kovacs GG, Ghetti B, Halliday G, Holm IE, Ince PG, Kamphorst W, Revesz T, Rozemuller AJM, Kumar-Singh S, Akiyama H, Baborie A, Spina S, Dickson DW, Trojanowski JQ, Mann DMA (2009) Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol 117:15\u201318","journal-title":"Acta Neuropathol"},{"key":"816_CR24","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00401-009-0612-2","volume":"119","author":"IR Mackenzie","year":"2010","unstructured":"Mackenzie IR, Neumann M, Bigio EH, Cairns NJ, Alafuzoff I, Kril J, Kovacs GG, Ghetti B, Halliday G, Holm IE, Ince PG, Kamphorst W, Revesz T, Rozemuller AJM, Kumar-Singh S, Akiyama H, Baborie A, Spina S, Dickson DW, Trojanowski JQ, Mann DMA (2010) Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations: an update. Acta Neuropathol 119:1\u20134","journal-title":"Acta Neuropathol"},{"key":"816_CR25","doi-asserted-by":"crossref","first-page":"1237","DOI":"10.1002\/mds.22555","volume":"24","author":"FJ Mateen","year":"2009","unstructured":"Mateen FJ, Josephs KA (2009) The clinical spectrum of stereotypies in frontotemporal lobar degeneration. Mov Disord 24:1237\u20131240","journal-title":"Mov Disord"},{"key":"816_CR26","doi-asserted-by":"crossref","unstructured":"Menon R, Baborie A, Jaros E, Mann DMA, Ray PS, Larner AJ (2010) What\u2019s in a name? Neuronal intermediate filament inclusion disease (NIFID), frontotemporal lobar degeneration-intermediate filament (FTLD-IF) or frontotemporal lobar degeneration-fused in sarcoma (FTLD-FUS)? J Neurol Neurosurg Psychiatry doi: 10.1136\/jnnp.2010.220947","DOI":"10.1136\/jnnp.2010.220947"},{"key":"816_CR27","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1001\/archneurol.2007.37","volume":"65","author":"L Molina-Porcel","year":"2008","unstructured":"Molina-Porcel L, Llado A, Rey MJ, Molinuevo JL, Martinez-Lage M, Esteve FX, Ferrer I, Tolosa E, Blesa R (2008) Clinical and pathological heterogeneity of neuronal intermediate filament inclusion disease. Arch Neurol 65:272\u2013275","journal-title":"Arch Neurol"},{"key":"816_CR28","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 IRA (2009) FUS pathology in basophilic inclusion body disease. Acta Neuropathol 118:617\u2013627","journal-title":"Acta Neuropathol"},{"key":"816_CR29","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1136\/jnnp.53.1.23","volume":"53","author":"D Neary","year":"1990","unstructured":"Neary D, Snowden JS, Mann DMA, Northen B, Goulding PJ, MacDermott N (1990) Frontal lobe dementia and motor neurone disease. J Neurol Neurosurg Psychiatry 53:23\u201332","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"816_CR30","doi-asserted-by":"crossref","first-page":"1546","DOI":"10.1212\/WNL.51.6.1546","volume":"51","author":"D Neary","year":"1998","unstructured":"Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546\u20131554","journal-title":"Neurology"},{"key":"816_CR31","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1016\/S1474-4422(05)70223-4","volume":"4","author":"D Neary","year":"2005","unstructured":"Neary D, Snowden JS, Mann DMA (2005) Frontotemporal dementia. Lancet Neurol 4:771\u2013780","journal-title":"Lancet Neurol"},{"key":"816_CR32","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 H, Trojanowski JQ, Lee VM (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130\u2013133","journal-title":"Science"},{"key":"816_CR33","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 IRA (2009) A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain 132:2922\u20132931","journal-title":"Brain"},{"key":"816_CR34","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, Kretzchmar HA, Rademakers R, Baker M, Mackenzie IRA (2009) Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol 118:605\u2013616","journal-title":"Acta Neuropathol"},{"key":"816_CR35","doi-asserted-by":"crossref","first-page":"1398","DOI":"10.1136\/jnnp.74.10.1398","volume":"74","author":"S Nyatsanza","year":"2003","unstructured":"Nyatsanza S, Shetty T, Gregory C, Lough S, Dawson K, Hodges JR (2003) A study of stereotypic behaviours in Alzheimer\u2019s disease and frontal and temporal variant frontotemporal dementia. J Neurol Neurosurg Psychiatry 74:1398\u20131402","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"816_CR36","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1093\/brain\/awf069","volume":"125","author":"SM Pickering-Brown","year":"2002","unstructured":"Pickering-Brown SM, Richardson AM, Snowden JS, McDonagh AM, Burns A, Braude W, Baker M, Liu W-K, Yen S-H, Hardy J, Hutton M, Davies Y, Allsop D, Craufurd D, Neary D, Mann DMA (2002) Inherited frontotemporal dementia in 9 British families associated with intronic mutations in the tau gene. Brain 125:732\u2013751","journal-title":"Brain"},{"key":"816_CR37","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1093\/brain\/awm331","volume":"131","author":"SM Pickering-Brown","year":"2008","unstructured":"Pickering-Brown SM, Rollinson S, Du Plessis D, Morrison KE, Varma A, Richardson AMT, Neary D, Snowden JS, Mann DMA (2008) Frequency and clinical characteristics of progranulin mutation carriers in the Manchester Frontotemporal Lobar Degeneration cohort: comparison to patients with MAPT and no known mutations. Brain 131:721\u2013731","journal-title":"Brain"},{"key":"816_CR38","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1212\/01.wnl.0000343004.98599.45","volume":"72","author":"O Piguet","year":"2009","unstructured":"Piguet O, Hornberger M, Shelley BP, Kipps CM, Hodges JR (2009) Sensitivity of current criteria for the diagnosis of behavioural variant frontotemporal dementia. Neurology 72:732\u2013737","journal-title":"Neurology"},{"key":"816_CR39","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1007\/s00401-008-0395-x","volume":"116","author":"S Roeber","year":"2008","unstructured":"Roeber S, Mackenzie IR, Kretzchmar HA, Neumann M (2008) TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD. Acta Neuropathol 116:147\u2013157","journal-title":"Acta Neuropathol"},{"key":"816_CR40","doi-asserted-by":"crossref","unstructured":"Rohrer JD, Lashley T, Holton J, Revesz T, Urwin H, Isaacs AM, Fox NC, Rossor MN, Warren J (2010) The clinical and neuroanatomical phenotype of FUS associated frontotemporal lobar degeneration. J Neurol Neurosurg Psychiatry doi: 10.1136\/jnnp.2010.214437","DOI":"10.1136\/jnnp.2010.214437"},{"key":"816_CR41","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.2353\/ajpath.2006.060438","volume":"169","author":"DM Sampathu","year":"2006","unstructured":"Sampathu DM, Neumann M, Kwong LK, Chou TT, Micsenyi M, Truax A, Bruce J, Grossman M, Trojanowski JQ, Lee VM (2006) Pathological heterogeneity of frontotemporal lobar degeneration with ubiquitin-positive inclusions delineated by ubiquitin immunohistochemistry and novel monoclonal antibodies. Am J Pathol 169:1343\u20131352","journal-title":"Am J Pathol"},{"key":"816_CR42","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1007\/s00415-009-5404-z","volume":"257","author":"H Seelaar","year":"2010","unstructured":"Seelaar H, Klijnsma KY, de Koning I, van der Lugt A, Zheng Chiu W, Azmani A, Rozemuller AJM, Van Swieten JC (2010) Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration. J Neurol 257:747\u2013753","journal-title":"J Neurol"},{"key":"816_CR43","volume-title":"Frontotemporal lobar degeneration: frontotemporal dementia progressive aphasia and semantic dementia","author":"JS Snowden","year":"1996","unstructured":"Snowden JS, Neary D, Mann DMA (1996) Frontotemporal lobar degeneration: frontotemporal dementia progressive aphasia and semantic dementia. Churchill Livingstone, London"},{"key":"816_CR44","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1136\/jnnp.70.3.323","volume":"70","author":"JS Snowden","year":"2001","unstructured":"Snowden JS, Bathgate B, Varma A, Blackshaw A, Gibbons ZC, Neary D (2001) Distinct behavioural profiles in frontotemporal dementia and semantic dementia. J Neurol Neurosurg Psychiatry 70:323\u2013332","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"816_CR45","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/s00401-007-0236-3","volume":"114","author":"JS Snowden","year":"2007","unstructured":"Snowden JS, Neary D, Mann DMA (2007) Frontotemporal lobar degeneration: clinical and pathological relationships. Acta Neuropathol 114:31\u201338","journal-title":"Acta Neuropathol"},{"key":"816_CR46","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.1086\/379378","volume":"73","author":"M Stephens","year":"2003","unstructured":"Stephens M, Donnelly P (2003) A comparison of bayesian methods for haplotype reconstruction from population genotype data. Am J Hum Genet 73:1162\u20131169","journal-title":"Am J Hum Genet"},{"key":"816_CR47","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1007\/s00415-003-0989-0","volume":"250","author":"J Stone","year":"2003","unstructured":"Stone J, Griffiths TD, Rastogi S, Perry RH, Cleland PG (2003) Non-Picks frontotemporal dementia imitating schizophrenia in a 22-year-old man. J Neurol 250:369\u2013370","journal-title":"J Neurol"},{"key":"816_CR48","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1080\/17482960802654364","volume":"10","author":"MJ Strong","year":"2009","unstructured":"Strong MJ, Grace GM, Freedman M, Lomen-Hoerth C, Woolley S, Goldstein LH, Murphy J, Shoesmith C, Rosenfeld J, Leigh PN, Bruijn L, Ince P, Figlewicz D (2009) Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 10:131\u2013146","journal-title":"Amyotroph Lateral Scler"},{"key":"816_CR49","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1007\/s00401-009-0621-1","volume":"119","author":"T Tateishi","year":"2010","unstructured":"Tateishi T, Hokonohara T, Yamasaki R, Miura S, Kikuchi H, Iwaki A, Tashiro H, Furuya H, Nagara Y, Ohyagi Y, Nukina N, Iwaki T, Fukumaki Y, Kira JI (2010) Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutation. Acta Neuropathol 119:355\u2013364","journal-title":"Acta Neuropathol"},{"key":"816_CR50","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, Consortium The FReJA, Dickson DW, Rademakers R, Graff-Radford NR, Parisi JE, Petersen RC, Hatanpaa KJ, White CL III, 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":"816_CR51","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1126\/science.1165942","volume":"323","author":"C Vance","year":"2009","unstructured":"Vance C, Roegelj 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":"816_CR52","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":"816_CR53","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1007\/s00401-007-0329-z","volume":"115","author":"O Yokota","year":"2008","unstructured":"Yokota O, Tsuchiya K, Terada S, Ishizu H, Uchikado H, Ikeda M, Oyanagi K, Nakazano I, Murayama S, Kuroda S, Akiyama H (2008) Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: a comparative clinicopathological study. Acta Neuropathol 115:561\u2013575","journal-title":"Acta Neuropathol"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-011-0816-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00401-011-0816-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-011-0816-0","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,6,9]],"date-time":"2019-06-09T06:17:05Z","timestamp":1560061025000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00401-011-0816-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,3,20]]},"references-count":53,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2011,7]]}},"alternative-id":["816"],"URL":"https:\/\/doi.org\/10.1007\/s00401-011-0816-0","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,3,20]]}}}