{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T05:34:46Z","timestamp":1789450486944,"version":"build-2803163510"},"reference-count":51,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,12,2]],"date-time":"2021-12-02T00:00:00Z","timestamp":1638403200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2021,12,2]],"date-time":"2021-12-02T00:00:00Z","timestamp":1638403200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"funder":[{"DOI":"10.13039\/501100001691","name":"japan society for the promotion of science","doi-asserted-by":"crossref","award":["201870008"],"award-info":[{"award-number":["201870008"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100001691","name":"japan society for the promotion of science","doi-asserted-by":"crossref","award":["201860169"],"award-info":[{"award-number":["201860169"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100008732","name":"uehara memorial foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008732","id-type":"DOI","asserted-by":"publisher"}]},{"name":"alzheimer\u2019s association research fellowship to promote diversity","award":["AARF-D-619473"],"award-info":[{"award-number":["AARF-D-619473"]}]},{"name":"rapid program in dementia","award":["AARF-D-619473-RAPID"],"award-info":[{"award-number":["AARF-D-619473-RAPID"]}]},{"DOI":"10.13039\/100000065","name":"national institute of neurological disorders and stroke","doi-asserted-by":"publisher","award":["K23-NS11416-01A1"],"award-info":[{"award-number":["K23-NS11416-01A1"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"national institute of neurological disorders and stroke","doi-asserted-by":"publisher","award":["P50 NS053488"],"award-info":[{"award-number":["P50 NS053488"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"national institute on aging","doi-asserted-by":"publisher","award":["P30 AG010124"],"award-info":[{"award-number":["P30 AG010124"]}],"id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"national institute on aging","doi-asserted-by":"publisher","award":["T32-AG000255"],"award-info":[{"award-number":["T32-AG000255"]}],"id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"national institute on aging","doi-asserted-by":"publisher","award":["P30 AG072979"],"award-info":[{"award-number":["P30 AG072979"]}],"id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"national institute on aging","doi-asserted-by":"publisher","award":["P01 AG066597"],"award-info":[{"award-number":["P01 AG066597"]}],"id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"national institute on aging","doi-asserted-by":"publisher","award":["U19 AG062418"],"award-info":[{"award-number":["U19 AG062418"]}],"id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]},{"name":"university of pennsylvania institute on aging"},{"DOI":"10.13039\/100017312","name":"lewy body dementia association","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100017312","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2022,1]]},"DOI":"10.1007\/s00401-021-02383-3","type":"journal-article","created":{"date-parts":[[2021,12,2]],"date-time":"2021-12-02T09:02:32Z","timestamp":1638435752000},"page":"15-31","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":57,"title":["Distinct characteristics of limbic-predominant age-related TDP-43 encephalopathy in Lewy body disease"],"prefix":"10.1007","volume":"143","author":[{"given":"Maiko T.","family":"Uemura","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John L.","family":"Robinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katheryn A. Q.","family":"Cousins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas F.","family":"Tropea","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel C.","family":"Kargilis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer D.","family":"McBride","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"EunRan","family":"Suh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon X.","family":"Xie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yan","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00edlvia","family":"Porta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norihito","family":"Uemura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivianna M.","family":"Van Deerlin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Wolk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David J.","family":"Irwin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kurt R.","family":"Brunden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginia M.-Y.","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward B.","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9239-8794","authenticated-orcid":false,"given":"John Q.","family":"Trojanowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,12,2]]},"reference":[{"key":"2383_CR1","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1002\/ana.21154","volume":"61","author":"C Amador-Ortiz","year":"2007","unstructured":"Amador-Ortiz C, Lin WL, Ahmed Z, Personett D, Davies P, Duara R et al (2007) TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer\u2019s disease. Ann Neurol 61:435\u2013445. https:\/\/doi.org\/10.1002\/ana.21154","journal-title":"Ann Neurol"},{"key":"2383_CR2","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1007\/s00401-014-1358-z","volume":"129","author":"N Aoki","year":"2015","unstructured":"Aoki N, Murray ME, Ogaki K, Fujioka S, Rutherford NJ, Rademakers R et al (2015) Hippocampal sclerosis in Lewy body disease is a TDP-43 proteinopathy similar to FTLD-TDP Type A. Acta Neuropathol 129:53\u201364. https:\/\/doi.org\/10.1007\/s00401-014-1358-z","journal-title":"Acta Neuropathol"},{"key":"2383_CR3","doi-asserted-by":"publisher","first-page":"305","DOI":"10.1093\/jnen\/nlz126","volume":"79","author":"LM Besser","year":"2020","unstructured":"Besser LM, Teylan MA, Nelson PT (2020) Limbic predominant age-related TDP-43 encephalopathy (LATE): clinical and neuropathological associations. J Neuropathol Exp Neurol 79:305\u2013313. https:\/\/doi.org\/10.1093\/jnen\/nlz126","journal-title":"J Neuropathol Exp Neurol"},{"key":"2383_CR4","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1016\/S0197-4580(02)00065-9PMID-12498954","volume":"24","author":"H Braak","year":"2003","unstructured":"Braak H, Tredici K, R\u00fcb U, Vos Rd, Steur E, Braak E (2003) Staging of brain pathology related to sporadic Parkinson\u2019s disease. Neurobiol Aging 24:197\u2013211. https:\/\/doi.org\/10.1016\/S0197-4580(02)00065-9PMID-12498954","journal-title":"Neurobiol Aging"},{"key":"2383_CR5","doi-asserted-by":"publisher","first-page":"294","DOI":"10.1038\/s41588-021-00785-3","volume":"53","author":"R Chia","year":"2021","unstructured":"Chia R, Sabir MS, Bandres-Ciga S, Saez-Atienzar S, Reynolds RH, Gustavsson E et al (2021) Genome sequencing analysis identifies new loci associated with Lewy body dementia and provides insights into its genetic architecture. Nat Genet 53:294\u2013303. https:\/\/doi.org\/10.1038\/s41588-021-00785-3","journal-title":"Nat Genet"},{"key":"2383_CR6","doi-asserted-by":"publisher","first-page":"843","DOI":"10.1002\/alz.12079","volume":"16","author":"R de Flores","year":"2020","unstructured":"de Flores R, Wisse LEM, Das SR, Xie L, McMillan CT, Trojanowski JQ et al (2020) Contribution of mixed pathology to medial temporal lobe atrophy in Alzheimer\u2019s disease. Alzheimers Dement 16:843\u2013852. https:\/\/doi.org\/10.1002\/alz.12079","journal-title":"Alzheimers Dement"},{"key":"2383_CR7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2021.166953","author":"S Dhakal","year":"2021","unstructured":"Dhakal S, Wyant CE, George HE, Morgan SE, Rangachari V (2021) Prion-like C-terminal domain of TDP-43 and \u03b1-Synuclein interact synergistically to generate neurotoxic hybrid fibrils. J Mol Biol. https:\/\/doi.org\/10.1016\/j.jmb.2021.166953","journal-title":"J Mol Biol"},{"key":"2383_CR8","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1159\/000289231","volume":"7","author":"DW Dickson","year":"2010","unstructured":"Dickson DW, Baker M, Rademakers R (2010) Common variant in GRN is a genetic risk factor for hippocampal sclerosis in the elderly. Neurodegener Dis 7:170\u2013174. https:\/\/doi.org\/10.1159\/000289231","journal-title":"Neurodegener Dis"},{"key":"2383_CR9","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/0022-3956(75)90026-6","volume":"12","author":"MF Folstein","year":"1975","unstructured":"Folstein MF, Folstein SE, McHugh PR (1975) \u201cMini-mental state\u201d. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189\u2013198. https:\/\/doi.org\/10.1016\/0022-3956(75)90026-6","journal-title":"J Psychiatr Res"},{"key":"2383_CR10","doi-asserted-by":"publisher","first-page":"407","DOI":"10.1007\/s00401-013-1239-x","volume":"127","author":"MD Gallagher","year":"2014","unstructured":"Gallagher MD, Suh E, Grossman M, Elman L, McCluskey L, Van Swieten JC et al (2014) TMEM106B is a genetic modifier of frontotemporal lobar degeneration with C9orf72 hexanucleotide repeat expansions. Acta Neuropathol 127:407\u2013418. https:\/\/doi.org\/10.1007\/s00401-013-1239-x","journal-title":"Acta Neuropathol"},{"key":"2383_CR11","doi-asserted-by":"publisher","first-page":"284","DOI":"10.1016\/j.brainres.2007.09.048","volume":"1184","author":"S Higashi","year":"2007","unstructured":"Higashi S, Iseki E, Yamamoto R, Minegishi M, Hino H, Fujisawa K et al (2007) Concurrence of TDP-43, tau and alpha-synuclein pathology in brains of Alzheimer\u2019s disease and dementia with Lewy bodies. Brain Res 1184:284\u2013294. https:\/\/doi.org\/10.1016\/j.brainres.2007.09.048","journal-title":"Brain Res"},{"key":"2383_CR12","doi-asserted-by":"publisher","first-page":"364","DOI":"10.1111\/bpa.12773","volume":"30","author":"SRK Hokkanen","year":"2020","unstructured":"Hokkanen SRK, Kero M, Kaivola K, Hunter S, Keage HAD, Kiviharju A et al (2020) Putative risk alleles for LATE-NC with hippocampal sclerosis in population-representative autopsy cohorts. Brain Pathol 30:364\u2013372. https:\/\/doi.org\/10.1111\/bpa.12773","journal-title":"Brain Pathol"},{"key":"2383_CR13","doi-asserted-by":"publisher","first-page":"917","DOI":"10.1016\/s1474-4422(17)30284-3","volume":"16","author":"KA Josephs","year":"2017","unstructured":"Josephs KA, Dickson DW, Tosakulwong N, Weigand SD, Murray ME, Petrucelli L et al (2017) Rates of hippocampal atrophy and presence of post-mortem TDP-43 in patients with Alzheimer\u2019s disease: a longitudinal retrospective study. Lancet Neurol 16:917\u2013924. https:\/\/doi.org\/10.1016\/s1474-4422(17)30284-3","journal-title":"Lancet Neurol"},{"key":"2383_CR14","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s00401-018-1951-7PMID-30604226","volume":"137","author":"KA Josephs","year":"2019","unstructured":"Josephs KA, Murray ME, Tosakulwong N, Weigand SD, Serie AM, Perkerson RB et al (2019) Pathological, imaging and genetic characteristics support the existence of distinct TDP-43 types in non-FTLD brains. Acta Neuropathol 137:1\u201312. https:\/\/doi.org\/10.1007\/s00401-018-1951-7PMID-30604226","journal-title":"Acta Neuropathol"},{"key":"2383_CR15","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1007\/s00401-013-1211-9","volume":"127","author":"KA Josephs","year":"2014","unstructured":"Josephs KA, Murray ME, Whitwell JL, Parisi JE, Petrucelli L, Jack CR et al (2014) Staging TDP-43 pathology in Alzheimer\u2019s disease. Acta Neuropathol 127:441\u2013450. https:\/\/doi.org\/10.1007\/s00401-013-1211-9","journal-title":"Acta Neuropathol"},{"key":"2383_CR16","doi-asserted-by":"publisher","first-page":"571","DOI":"10.1007\/s00401-016-1537-1","volume":"131","author":"KA Josephs","year":"2016","unstructured":"Josephs KA, Murray ME, Whitwell JL, Tosakulwong N, Weigand SD, Petrucelli L et al (2016) Updated TDP-43 in Alzheimer\u2019s disease staging scheme. Acta Neuropathol 131:571\u2013585. https:\/\/doi.org\/10.1007\/s00401-016-1537-1","journal-title":"Acta Neuropathol"},{"key":"2383_CR17","doi-asserted-by":"publisher","first-page":"697","DOI":"10.1002\/ana.24493","volume":"78","author":"KA Josephs","year":"2015","unstructured":"Josephs KA, Whitwell JL, Tosakulwong N, Weigand SD, Murray ME, Liesinger AM et al (2015) TAR DNA-binding protein 43 and pathological subtype of Alzheimer\u2019s disease impact clinical features. Ann Neurol 78:697\u2013709. https:\/\/doi.org\/10.1002\/ana.24493","journal-title":"Ann Neurol"},{"key":"2383_CR18","doi-asserted-by":"publisher","first-page":"1299","DOI":"10.1001\/jamaneurol.2020.1741","volume":"77","author":"S Karanth","year":"2020","unstructured":"Karanth S, Nelson PT, Katsumata Y, Kryscio RJ, Schmitt FA, Fardo DW et al (2020) Prevalence and clinical phenotype of quadruple misfolded proteins in older adults. JAMA Neurol 77:1299\u20131307. https:\/\/doi.org\/10.1001\/jamaneurol.2020.1741","journal-title":"JAMA Neurol"},{"key":"2383_CR19","doi-asserted-by":"publisher","first-page":"659","DOI":"10.1007\/s00401-020-02211-0","volume":"140","author":"Y Katsumata","year":"2020","unstructured":"Katsumata Y, Abner EL, Karanth S, Teylan MA, Mock CN, Cykowski MD et al (2020) Distinct clinicopathologic clusters of persons with TDP-43 proteinopathy. Acta Neuropathol 140:659\u2013674. https:\/\/doi.org\/10.1007\/s00401-020-02211-0","journal-title":"Acta Neuropathol"},{"key":"2383_CR20","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1186\/s40478-018-0641-y","volume":"6","author":"Y Katsumata","year":"2018","unstructured":"Katsumata Y, Fardo DW, Kukull WA, Nelson PT (2018) Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer\u2019s disease and cerebrovascular disease pathologies. Acta Neuropathol Commun 6:142. https:\/\/doi.org\/10.1186\/s40478-018-0641-y","journal-title":"Acta Neuropathol Commun"},{"key":"2383_CR21","doi-asserted-by":"publisher","first-page":"50","DOI":"10.1186\/s40478-018-0552-y","volume":"6","author":"GG Kovacs","year":"2018","unstructured":"Kovacs GG, Xie SX, Robinson JL, Lee EB, Smith DH, Schuck T et al (2018) Sequential stages and distribution patterns of aging-related tau astrogliopathy (ARTAG) in the human brain. Acta Neuropathol Commun 6:50. https:\/\/doi.org\/10.1186\/s40478-018-0552-y","journal-title":"Acta Neuropathol Commun"},{"key":"2383_CR22","doi-asserted-by":"publisher","first-page":"472","DOI":"10.1111\/bpa.12424","volume":"27","author":"KE McAleese","year":"2017","unstructured":"McAleese KE, Walker L, Erskine D, Thomas AJ, McKeith IG, Attems J (2017) TDP-43 pathology in Alzheimer\u2019s disease, dementia with Lewy bodies and ageing. Brain Pathol 27:472\u2013479. https:\/\/doi.org\/10.1111\/bpa.12424","journal-title":"Brain Pathol"},{"key":"2383_CR23","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1212\/WNL.0000000000004058","volume":"89","author":"IG McKeith","year":"2017","unstructured":"McKeith IG, Boeve BF, Dickson DW, Halliday G, Taylor J-P, Weintraub D et al (2017) Diagnosis and management of dementia with Lewy bodies: fourth consensus report of the DLB consortium. Neurology 89:88\u2013100. https:\/\/doi.org\/10.1212\/WNL.0000000000004058","journal-title":"Neurology"},{"key":"2383_CR24","doi-asserted-by":"publisher","first-page":"1863","DOI":"10.1212\/01.wnl.0000187889.17253.b1PMID-16237129","volume":"65","author":"IG McKeith","year":"2005","unstructured":"McKeith IG, Dickson DW, Lowe J, Emre M, O\u2019Brien JT, Feldman H et al (2005) Diagnosis and management of dementia with Lewy bodies: third report of the DLB consortium. Neurology 65:1863\u20131872. https:\/\/doi.org\/10.1212\/01.wnl.0000187889.17253.b1PMID-16237129","journal-title":"Neurology"},{"key":"2383_CR25","doi-asserted-by":"publisher","first-page":"1473","DOI":"10.1016\/j.neurobiolaging.2013.11.029","volume":"35","author":"CT McMillan","year":"2014","unstructured":"McMillan CT, Toledo JB, Avants BB, Cook PA, Wood EM, Suh E et al (2014) Genetic and neuroanatomic associations in sporadic frontotemporal lobar degeneration. Neurobiol Aging 35:1473\u20131482. https:\/\/doi.org\/10.1016\/j.neurobiolaging.2013.11.029","journal-title":"Neurobiol Aging"},{"key":"2383_CR26","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2020.105130","volume":"146","author":"M Montalbano","year":"2020","unstructured":"Montalbano M, McAllen S, Cascio FL, Sengupta U, Garcia S, Bhatt N et al (2020) TDP-43 and Tau oligomers in Alzheimer\u2019s disease, amyotrophic lateral sclerosis, and frontotemporal dementia. Neurobiol Dis 146:105130. https:\/\/doi.org\/10.1016\/j.nbd.2020.105130","journal-title":"Neurobiol Dis"},{"key":"2383_CR27","doi-asserted-by":"publisher","first-page":"164","DOI":"10.1016\/j.jalz.2015.07.492","volume":"12","author":"TJ Montine","year":"2016","unstructured":"Montine TJ, Monsell SE, Beach TG, Bigio EH, Bu Y, Cairns NJ et al (2016) Multisite assessment of NIA-AA guidelines for the neuropathologic evaluation of Alzheimer\u2019s disease. J lzheimer\u2019s Dementia Assoc 12:164\u2013169. https:\/\/doi.org\/10.1016\/j.jalz.2015.07.492","journal-title":"J lzheimer's Dementia Assoc"},{"key":"2383_CR28","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s00401-011-0910-3","volume":"123","author":"TJ Montine","year":"2012","unstructured":"Montine TJ, Phelps CH, Beach TG, Bigio EH, Cairns NJ, Dickson DW et al (2012) National institute on Aging-Alzheimer\u2019s association guidelines for the neuropathologic assessment of Alzheimer\u2019s disease: a practical approach. Acta Neuropathol 123:1\u201311. https:\/\/doi.org\/10.1007\/s00401-011-0910-3","journal-title":"Acta Neuropathol"},{"key":"2383_CR29","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1007\/s00401-014-1302-2","volume":"128","author":"ME Murray","year":"2014","unstructured":"Murray ME, Cannon A, Graff-Radford NR, Liesinger AM, Rutherford NJ, Ross OA et al (2014) Differential clinicopathologic and genetic features of late-onset amnestic dementias. Acta Neuropathol 128:411\u2013421. https:\/\/doi.org\/10.1007\/s00401-014-1302-2","journal-title":"Acta Neuropathol"},{"key":"2383_CR30","doi-asserted-by":"publisher","first-page":"942","DOI":"10.1002\/ana.24388","volume":"77","author":"S Nag","year":"2015","unstructured":"Nag S, Yu L, Capuano AW, Wilson RS, Leurgans SE, Bennett DA et al (2015) Hippocampal sclerosis and TDP-43 pathology in aging and Alzheimer disease. Ann Neurol 77:942\u2013952. https:\/\/doi.org\/10.1002\/ana.24388","journal-title":"Ann Neurol"},{"key":"2383_CR31","doi-asserted-by":"publisher","first-page":"606","DOI":"10.1111\/neup.12673","volume":"40","author":"K Nakamura-Shindo","year":"2020","unstructured":"Nakamura-Shindo K, Sakai K, Shimizu A, Ishida C, Yamada M (2020) Accumulation of phosphorylated TDP-43 in the cytoplasm of Schwann cells in a case of sporadic amyotrophic lateral sclerosis. Neuropathology 40:606\u2013610. https:\/\/doi.org\/10.1111\/neup.12673","journal-title":"Neuropathology"},{"key":"2383_CR32","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1007\/s00401-007-0261-2","volume":"114","author":"H Nakashima-Yasuda","year":"2007","unstructured":"Nakashima-Yasuda H, Uryu K, Robinson J, Xie SX, Hurtig H, Duda JE et al (2007) Co-morbidity of TDP-43 proteinopathy in Lewy body related diseases. Acta Neuropathol 114:221\u2013229. https:\/\/doi.org\/10.1007\/s00401-007-0261-2","journal-title":"Acta Neuropathol"},{"key":"2383_CR33","doi-asserted-by":"publisher","first-page":"362","DOI":"10.1097\/NEN.0b013e31825018f7","volume":"71","author":"PT Nelson","year":"2012","unstructured":"Nelson PT, Alafuzoff I, Bigio EH, Bouras C, Braak H, Cairns NJ et al (2012) Correlation of Alzheimer disease neuropathologic changes with cognitive status: a review of the literature. J Neuropathol Exp Neurol 71:362\u2013381. https:\/\/doi.org\/10.1097\/NEN.0b013e31825018f7","journal-title":"J Neuropathol Exp Neurol"},{"key":"2383_CR34","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1097\/NEN.0b013e3181919a48","volume":"68","author":"PT Nelson","year":"2009","unstructured":"Nelson PT, Braak H, Markesbery WR (2009) Neuropathology and cognitive impairment in Alzheimer disease: a complex but coherent relationship. J Neuropathol Exp Neurol 68:1\u201314. https:\/\/doi.org\/10.1097\/NEN.0b013e3181919a48","journal-title":"J Neuropathol Exp Neurol"},{"key":"2383_CR35","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.1093\/brain\/awz099","volume":"142","author":"PT Nelson","year":"2019","unstructured":"Nelson PT, Dickson DW, Trojanowski JQ, Jack CR, Boyle PA, Arfanakis K et al (2019) Limbic-predominant age-related TDP-43 encephalopathy (LATE): consensus working group report. Brain 142:1503\u20131527. https:\/\/doi.org\/10.1093\/brain\/awz099","journal-title":"Brain"},{"key":"2383_CR36","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1097\/nen.0000000000000151","volume":"74","author":"PT Nelson","year":"2015","unstructured":"Nelson PT, Wang WX, Partch AB, Monsell SE, Valladares O, Ellingson SR et al (2015) Reassessment of risk genotypes (GRN, TMEM106B, and ABCC9 variants) associated with hippocampal sclerosis of aging pathology. J Neuropathol Exp Neurol 74:75\u201384. https:\/\/doi.org\/10.1097\/nen.0000000000000151","journal-title":"J Neuropathol Exp Neurol"},{"key":"2383_CR37","doi-asserted-by":"publisher","first-page":"255","DOI":"10.1093\/brain\/awt318","volume":"137","author":"JH Neltner","year":"2014","unstructured":"Neltner JH, Abner EL, Baker S, Schmitt FA, Kryscio RJ, Jicha GA et al (2014) Arteriolosclerosis that affects multiple brain regions is linked to hippocampal sclerosis of ageing. Brain 137:255\u2013267. https:\/\/doi.org\/10.1093\/brain\/awt318","journal-title":"Brain"},{"key":"2383_CR38","doi-asserted-by":"publisher","first-page":"645","DOI":"10.1007\/s00401-020-02207-wPMID-32778941","volume":"140","author":"M Neumann","year":"2020","unstructured":"Neumann M, Frick P, Paron F, Kosten J, Buratti E, Mackenzie IR (2020) Antibody against TDP-43 phosphorylated at serine 375 suggests conformational differences of TDP-43 aggregates among FTLD\u2013TDP subtypes. Acta Neuropathol 140:645\u2013658. https:\/\/doi.org\/10.1007\/s00401-020-02207-wPMID-32778941","journal-title":"Acta Neuropathol"},{"key":"2383_CR39","doi-asserted-by":"publisher","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 et al (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130\u2013133. https:\/\/doi.org\/10.1126\/science.1134108","journal-title":"Science"},{"key":"2383_CR40","doi-asserted-by":"publisher","first-page":"172","DOI":"10.1038\/jcbfm.2015.164","volume":"36","author":"L Raz","year":"2016","unstructured":"Raz L, Knoefel J, Bhaskar K (2016) The neuropathology and cerebrovascular mechanisms of dementia. J Cereb Blood Flow Metab 36:172\u2013186. https:\/\/doi.org\/10.1038\/jcbfm.2015.164","journal-title":"J Cereb Blood Flow Metab"},{"key":"2383_CR41","doi-asserted-by":"publisher","first-page":"2181","DOI":"10.1093\/brain\/awy146","volume":"141","author":"JL Robinson","year":"2018","unstructured":"Robinson JL, Lee EB, Xie SX, Rennert L, Suh E, Bredenberg C et al (2018) Neurodegenerative disease concomitant proteinopathies are prevalent, age-related and APOE4-associated. Brain 141:2181\u20132193. https:\/\/doi.org\/10.1093\/brain\/awy146","journal-title":"Brain"},{"key":"2383_CR42","doi-asserted-by":"publisher","first-page":"awaa219","DOI":"10.1093\/brain\/awaa219","volume":"143","author":"JL Robinson","year":"2020","unstructured":"Robinson JL, Porta S, Garrett FG, Zhang P, Xie SX, Suh E et al (2020) Limbic-predominant age-related TDP-43 encephalopathy differs from frontotemporal lobar degeneration. Brain 143:awaa219. https:\/\/doi.org\/10.1093\/brain\/awaa219","journal-title":"Brain"},{"key":"2383_CR43","doi-asserted-by":"publisher","first-page":"264","DOI":"10.1111\/bpa.12505","volume":"28","author":"VD Smith","year":"2018","unstructured":"Smith VD, Bachstetter AD, Ighodaro E, Roberts K, Abner EL, Fardo DW (2018) Overlapping but distinct TDP-43 and tau pathologic patterns in aged hippocampi. Brain Pathol 28:264\u2013273. https:\/\/doi.org\/10.1111\/bpa.12505","journal-title":"Brain Pathol"},{"key":"2383_CR44","doi-asserted-by":"publisher","first-page":"234","DOI":"10.7150\/ijbs.7.234","volume":"7","author":"T Tian","year":"2011","unstructured":"Tian T, Huang C, Tong J, Yang M, Zhou H, Xia XG (2011) TDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic mice. Int J Biol Sci 7:234\u2013243. https:\/\/doi.org\/10.7150\/ijbs.7.234","journal-title":"Int J Biol Sci"},{"key":"2383_CR45","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1016\/j.jalz.2013.06.003","volume":"10","author":"JB Toledo","year":"2014","unstructured":"Toledo JB, Van Deerlin VM, Lee EB, Suh E, Baek Y, Robinson JL et al (2014) A platform for discovery: the University of Pennsylvania integrated neurodegenerative disease biobank. Alzheimers Dement 10:477-484.e471. https:\/\/doi.org\/10.1016\/j.jalz.2013.06.003","journal-title":"Alzheimers Dement"},{"key":"2383_CR46","doi-asserted-by":"publisher","first-page":"795","DOI":"10.1007\/s00401-021-02295-2","volume":"141","author":"SO Tom\u00e9","year":"2021","unstructured":"Tom\u00e9 SO, Gomes LA, Li X, Vandenberghe R, Tousseyn T, Thal DR (2021) TDP-43 interacts with pathological \u03c4 protein in Alzheimer\u2019s disease. Acta Neuropathol 141:795\u2013799. https:\/\/doi.org\/10.1007\/s00401-021-02295-2","journal-title":"Acta Neuropathol"},{"key":"2383_CR47","doi-asserted-by":"publisher","first-page":"801","DOI":"10.1002\/ana.25486PMID-30973966","volume":"85","author":"TF Tropea","year":"2019","unstructured":"Tropea TF, Mak J, Guo MH, Xie SX, Suh E, Rick J et al (2019) TMEM106B effect on cognition in Parkinson disease and frontotemporal dementia. Ann Neurol 85:801\u2013811. https:\/\/doi.org\/10.1002\/ana.25486PMID-30973966","journal-title":"Ann Neurol"},{"key":"2383_CR48","doi-asserted-by":"publisher","first-page":"234","DOI":"10.1038\/ng.536","volume":"42","author":"VM Van Deerlin","year":"2010","unstructured":"Van Deerlin VM, Sleiman PM, Martinez-Lage M, Chen-Plotkin A, Wang LS, Graff-Radford NR et al (2010) Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Nat Genet 42:234\u2013239. https:\/\/doi.org\/10.1038\/ng.536","journal-title":"Nat Genet"},{"key":"2383_CR49","doi-asserted-by":"publisher","first-page":"1683","DOI":"10.1016\/S0140-6736(15)00462-6","volume":"386","author":"Z Walker","year":"2015","unstructured":"Walker Z, Possin KL, Boeve BF, Aarsland D (2015) Lewy body dementias. Lancet (London, England) 386:1683\u20131697. https:\/\/doi.org\/10.1016\/S0140-6736(15)00462-6","journal-title":"Lancet (London, England)"},{"key":"2383_CR50","doi-asserted-by":"publisher","first-page":"e84","DOI":"10.1016\/j.jalz.2010.08.233","volume":"7","author":"SX Xie","year":"2011","unstructured":"Xie SX, Baek Y, Grossman M, Arnold SE, Karlawish J, Siderowf A et al (2011) Building an integrated neurodegenerative disease database at an academic health center. Alzheimers Dement 7:e84\u2013e93. https:\/\/doi.org\/10.1016\/j.jalz.2010.08.233","journal-title":"Alzheimers Dement"},{"key":"2383_CR51","doi-asserted-by":"publisher","first-page":"927","DOI":"10.1212\/wnl.0000000000001313","volume":"84","author":"L Yu","year":"2015","unstructured":"Yu L, De Jager PL, Yang J, Trojanowski JQ, Bennett DA, Schneider JA (2015) The TMEM106B locus and TDP-43 pathology in older persons without FTLD. Neurology 84:927\u2013934. https:\/\/doi.org\/10.1212\/wnl.0000000000001313","journal-title":"Neurology"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-021-02383-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00401-021-02383-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-021-02383-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,5]],"date-time":"2022-01-05T11:12:27Z","timestamp":1641381147000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00401-021-02383-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12,2]]},"references-count":51,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,1]]}},"alternative-id":["2383"],"URL":"https:\/\/doi.org\/10.1007\/s00401-021-02383-3","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,12,2]]},"assertion":[{"value":"5 August 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 November 2021","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 November 2021","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 December 2021","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"DAW reports grants from Merck, Biogen, and Avid\/Eli Lilly. He has received personal fees from GE Healthcare, Functional Neuromodulation, and Neuronix. EBL and JQT are members of Acta Neuropathologica\u2019s Editorial Board but were not involved in the editorial handling of this article.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}