{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T11:57:34Z","timestamp":1788955054925,"version":"build-2803163510"},"reference-count":83,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2021,1,9]],"date-time":"2021-01-09T00:00:00Z","timestamp":1610150400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Fundaci\u00f3 Miquel Valls"},{"name":"Retos Todos Unidos Contra la ELA"},{"name":"Proyecto DGeneracion Conexiones con Sentido"},{"DOI":"10.13039\/501100003329","name":"Ministry of Economy and Competitiveness","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003329","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Institute of Health Carlos III","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008530","name":"ERDF","doi-asserted-by":"publisher","award":["FISPI17\/000809"],"award-info":[{"award-number":["FISPI17\/000809"]}],"id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,2,22]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Sporadic amyotrophic lateral sclerosis (sALS) and FTLD-TDP are neurodegenerative diseases within the spectrum of TDP-43 proteinopathies. Since abnormal blood vessels and altered blood-brain barrier have been described in sALS, we wanted to know whether TDP-43 pathology also occurs in blood vessels in sALS\/FTLD-TDP. TDP-43 deposits were identified in association with small blood vessels of the spinal cord in 7 of 14 cases of sALS and in small blood vessels of frontal cortex area 8 in 6 of 11 FTLD-TDP and sALS cases, one of them carrying a GRN mutation. This was achieved using single and double-labeling immunohistochemistry, and double-labeling immunofluorescence and confocal microscopy. In the sALS spinal cord, P-TDP43 Ser403-404 deposits were elongated and parallel to the lumen, whereas others were granular, seldom forming clusters. In the frontal cortex, the inclusions were granular, or elongated and parallel to the lumen, or forming small globules within or in the external surface of the blood vessel wall. Other deposits were localized in the perivascular space. The present findings are in line with previous observations of TDP-43 vasculopathy in a subset of FTLD-TDP cases and identify this pathology in the spinal cord and frontal cortex in a subset of cases within the sALS\/FTLD-TDP spectrum.<\/jats:p>","DOI":"10.1093\/jnen\/nlaa162","type":"journal-article","created":{"date-parts":[[2021,2,22]],"date-time":"2021-02-22T17:21:13Z","timestamp":1614014473000},"page":"229-239","source":"Crossref","is-referenced-by-count":28,"title":["TDP-43 Vasculopathy in the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis (sALS) and Frontal Cortex in sALS\/FTLD-TDP"],"prefix":"10.1093","volume":"80","author":[{"given":"Isidro","family":"Ferrer","sequence":"first","affiliation":[{"name":"From the Department of Pathology and Experimental Therapeutics, University of Barcelona, L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"Biomedical Network Research Center on Neurodegenerative Diseases (CIBERNED), Institute Carlos III, L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"Bellvitge Biomedical Research Institute (IDIBELL), L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"Institute of Neurosciences, University of Barcelona , Barcelona, Spain"},{"name":"Neuropathology, Pathologic Anatomy Service, Bellvitge University Hospital, L\u2019Hospitalet de Llobregat , Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pol","family":"Andr\u00e9s-Benito","sequence":"additional","affiliation":[{"name":"From the Department of Pathology and Experimental Therapeutics, University of Barcelona, L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"Biomedical Network Research Center on Neurodegenerative Diseases (CIBERNED), Institute Carlos III, L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"Bellvitge Biomedical Research Institute (IDIBELL), L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"Institute of Neurosciences, University of Barcelona , Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarita","family":"Carmona","sequence":"additional","affiliation":[{"name":"From the Department of Pathology and Experimental Therapeutics, University of Barcelona, L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"Biomedical Network Research Center on Neurodegenerative Diseases (CIBERNED), Institute Carlos III, L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"Bellvitge Biomedical Research Institute (IDIBELL), L\u2019Hospitalet de Llobregat , Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdelilah","family":"Assialioui","sequence":"additional","affiliation":[{"name":"Functional Unit of Amyotrophic Lateral Sclerosis (UFELA), Service of Neurology, Bellvitge University Hospital, L\u2019Hospitalet de Llobregat , Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00f3nica","family":"Povedano","sequence":"additional","affiliation":[{"name":"Functional Unit of Amyotrophic Lateral Sclerosis (UFELA), Service of Neurology, Bellvitge University Hospital, L\u2019Hospitalet de Llobregat , Barcelona, Spain"},{"name":"International Initiative for Treatment and Research Initiative to Cure ALS (TRICALS) , Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,1,9]]},"reference":[{"key":"2024032523375850500_nlaa162-B1","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1097\/01.jnen.0000174335.27708.0a","article-title":"Ubiquitin immunohistochemistry suggests classic motor neuron disease, motor neuron disease with dementia, and frontotemporal dementia of the motor neuron disease type represent a clinicopathologic spectrum","volume":"64","author":"Mackenzie","year":"2005","journal-title":"J Neuropathol Exp Neurol"},{"key":"2024032523375850500_nlaa162-B2","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":"2024032523375850500_nlaa162-B3","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1007\/s00401-007-0315-5","article-title":"Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease","volume":"115","author":"Brandmeir","year":"2007","journal-title":"Acta Neuropathol"},{"key":"2024032523375850500_nlaa162-B4","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1001\/archneurol.2008.558","article-title":"Clinical and pathological continuum of multisystem TDP-43 proteinopathies","volume":"66","author":"Geser","year":"2009","journal-title":"Arch Neurol"},{"key":"2024032523375850500_nlaa162-B5","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1002\/9781444341256.ch44","volume-title":"Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders","author":"Strong","year":"2011","edition":"2"},{"key":"2024032523375850500_nlaa162-B6","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1159\/000109762","article-title":"Motor neuron disease and frontotemporal lobar degeneration: A tale of two disorders linked to TDP-43","volume":"16","author":"Elman","year":"2008","journal-title":"Neurosignals"},{"key":"2024032523375850500_nlaa162-B7","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1016\/j.pneurobio.2011.08.011","article-title":"On the development of markers for pathological TDP-43 in amyotrophic lateral sclerosis with and without dementia","volume":"95","author":"Geser","year":"2011","journal-title":"Prog Neurobiol"},{"key":"2024032523375850500_nlaa162-B8","first-page":"209","volume-title":"Neuropathology of Neurodegenerative Diseases: A Practical Guide","author":"Hortobagyi","year":"2015"},{"key":"2024032523375850500_nlaa162-B9","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1093\/jnen\/nlx063","article-title":"Amygdala TDP43 pathology in frontotemporal degeneration and motor neuron disease","volume":"76","author":"Takeda","year":"2017","journal-title":"J Neuropathol Exp Neurol"},{"key":"2024032523375850500_nlaa162-B10","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":"2024032523375850500_nlaa162-B11","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1523\/JNEUROSCI.4988-09.2010","article-title":"Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis","volume":"30","author":"Barmada","year":"2010","journal-title":"J. Neurosci"},{"key":"2024032523375850500_nlaa162-B12","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1007\/s00401-012-1022-4","article-title":"The genetics and neuropathology of amyotrophic lateral sclerosis","volume":"124","author":"Al-Chalabi","year":"2012","journal-title":"Acta Neuropathol"},{"key":"2024032523375850500_nlaa162-B13","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1097\/01.jnen.0000248554.45456.58","article-title":"TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions","volume":"66","author":"Neumann","year":"2007","journal-title":"J Neuropathol Exp Neurol"},{"key":"2024032523375850500_nlaa162-B14","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1007\/s00401-008-0396-9","article-title":"Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia","volume":"116","author":"Mori","year":"2008","journal-title":"Acta Neuropathol"},{"key":"2024032523375850500_nlaa162-B15","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1007\/s00401-006-0138-9","article-title":"Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: Classification and relation to clinical phenotype","volume":"112","author":"Mackenzie","year":"2006","journal-title":"Acta Neuropathol"},{"key":"2024032523375850500_nlaa162-B16","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.2353\/ajpath.2006.060438","article-title":"Pathological heterogeneity of frontotemporal lobar degeneration with ubiquitin-positive inclusions delineated by ubiquitin immunohistochemistry and novel monoclonal antibodies","volume":"169","author":"Sampathu","year":"2006","journal-title":"Am J Pathol"},{"key":"2024032523375850500_nlaa162-B17","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":"2024032523375850500_nlaa162-B18","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1186\/2051-5960-1-33","article-title":"Classification of FTLD-TDP cases into pathological subtypes using antibodies against phosphorylated and non-phosphorylated TDP43","volume":"1","author":"Tan","year":"2013","journal-title":"Acta Neuropathol Commun"},{"key":"2024032523375850500_nlaa162-B19","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":"2024032523375850500_nlaa162-B20","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1007\/s00401-006-0189-y","article-title":"Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43","volume":"113","author":"Davidson","year":"2007","journal-title":"Acta Neuropathol"},{"key":"2024032523375850500_nlaa162-B21","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/s00401-007-0234-5","article-title":"TDP-43 in the differential diagnosis of motor neuron disorders","volume":"114","author":"Dickson","year":"2007","journal-title":"Acta Neuropathol"},{"key":"2024032523375850500_nlaa162-B22","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.jns.2007.12.016","article-title":"Anterior horn cells with abnormal TDP-43 immunoreactivities show fragmentation of the Golgi apparatus in ALS","volume":"269","author":"Fujita","year":"2008","journal-title":"J Neurol Sci"},{"key":"2024032523375850500_nlaa162-B23","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1126\/science.1154584","article-title":"TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis","volume":"319","author":"Sreedharan","year":"2008","journal-title":"Science"},{"key":"2024032523375850500_nlaa162-B24","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1136\/jmg.2010.077180","article-title":"SOD1, ANG, VAPB, TARDBP, and FUS mutations in familial amyotrophic lateral sclerosis: Genotype-phenotype correlations","volume":"47","author":"Millecamps","year":"2010","journal-title":"J Med Genet"},{"key":"2024032523375850500_nlaa162-B25","doi-asserted-by":"crossref","first-page":"331","DOI":"10.2169\/internalmedicine.49.2915","article-title":"TDP-43 M337V mutation in familial amyotrophic lateral sclerosis in Japan","volume":"49","author":"Tamaoka","year":"2010","journal-title":"Intern Med"},{"key":"2024032523375850500_nlaa162-B26","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1186\/1471-2350-11-8","article-title":"Association between novel TARDBP mutations and Chinese patients with amyotrophic lateral sclerosis","volume":"11","author":"Xiong","year":"2010","journal-title":"BMC Med Genet"},{"key":"2024032523375850500_nlaa162-B27","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1007\/s11910-016-0658-1","article-title":"ALS: Recent developments from genetics studies","volume":"16","author":"Therrien","year":"2016","journal-title":"Curr Neurol Neurosci Rep"},{"key":"2024032523375850500_nlaa162-B28","doi-asserted-by":"crossref","first-page":"2111","DOI":"10.1007\/s10072-019-03943-y","article-title":"A novel S379A TARDBP mutation associated to late-onset sporadic ALS","volume":"40","author":"Sprovieri","year":"2019","journal-title":"Neurol Sci"},{"key":"2024032523375850500_nlaa162-B29","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":"118","author":"Gitcho","year":"2009","journal-title":"Acta Neuropathol"},{"key":"2024032523375850500_nlaa162-B30","doi-asserted-by":"crossref","first-page":"1843","DOI":"10.1002\/mds.22697","article-title":"TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea","volume":"24","author":"Kovacs","year":"2009","journal-title":"Mov Disord"},{"key":"2024032523375850500_nlaa162-B31","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1111\/nan.12063","article-title":"TARDBP mutation p.Ile383Val associated with semantic dementia and complex proteinopathy","volume":"40","author":"Gelpi","year":"2014","journal-title":"Neuropathol Appl Neurobiol"},{"key":"2024032523375850500_nlaa162-B32","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1186\/s40478-015-0190-6","article-title":"A novel mutation P112H in the TARDBP gene associated with frontotemporal lobar degeneration without motor neuron disease and abundant neuritic amyloid plaques","volume":"3","author":"Moreno","year":"2015","journal-title":"Acta Neuropathol Commun"},{"key":"2024032523375850500_nlaa162-B33","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/j.1365-2990.2003.00543.x","article-title":"Immunohistochemical localization of vascular endothelial growth factor receptors-1, -2 and -3 in the human spinal cord: Altered expression in amyotrophic lateral sclerosis","volume":"30","author":"Spliet","year":"2004","journal-title":"Neuropathol Appl Neurobiol"},{"key":"2024032523375850500_nlaa162-B34","doi-asserted-by":"crossref","first-page":"1614","DOI":"10.1212\/WNL.0b013e3181a41228","article-title":"Decreased mRNA expression of tight junction proteins in lumbar spinal cords of patients with ALS","volume":"72","author":"Henkel","year":"2009","journal-title":"Neurology"},{"key":"2024032523375850500_nlaa162-B35","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.brainres.2011.04.049","article-title":"Amyotrophic lateral sclerosis: A neurovascular disease","volume":"1398","author":"Garbuzova-Davis","year":"2011","journal-title":"Brain Res"},{"key":"2024032523375850500_nlaa162-B36","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.brainres.2012.05.056","article-title":"Impaired blood-brain\/spinal cord barrier in ALS patients","volume":"1469","author":"Garbuzova-Davis","year":"2012","journal-title":"Brain Res"},{"key":"2024032523375850500_nlaa162-B37","doi-asserted-by":"crossref","first-page":"1541","DOI":"10.1007\/s10072-019-03877-5","article-title":"The potential roles of aquaporin 4 in amyotrophic lateral sclerosis","volume":"40","author":"Zou","year":"2019","journal-title":"Neurol Sci"},{"key":"2024032523375850500_nlaa162-B38","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1007\/s00401-012-1039-8","article-title":"Blood-spinal cord barrier breakdown and pericyte reductions in amyotrophic lateral sclerosis","volume":"125","author":"Winkler","year":"2013","journal-title":"Acta Neuropathol"},{"key":"2024032523375850500_nlaa162-B39","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1111\/neup.12221","article-title":"Alterations of the blood-spinal cord barrier in sporadic amyotrophic lateral sclerosis","volume":"35","author":"Sasaki","year":"2015","journal-title":"Neuropathology"},{"key":"2024032523375850500_nlaa162-B40","doi-asserted-by":"crossref","first-page":"393","DOI":"10.3109\/21678421.2015.1011663","article-title":"Microvascular disturbance with decreased pericyte coverage is prominent in the ventral horn of patients with amyotrophic lateral sclerosis","volume":"16","author":"Yamadera","year":"2015","journal-title":"Amyotr Lat Scler Frontotemp Deg"},{"key":"2024032523375850500_nlaa162-B41","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/0165-5728(84)90042-0","article-title":"Deposits of IgG and C3 in the spinal cord and motor cortex of ALS patients","volume":"6","author":"Donnenfeld","year":"1984","journal-title":"J Neuroimmunol"},{"key":"2024032523375850500_nlaa162-B42","doi-asserted-by":"crossref","first-page":"1210","DOI":"10.1001\/archneur.1990.00530110068019","article-title":"IgG reactivity in the spinal cord and motor cortex in amyotrophic lateral sclerosis","volume":"47","author":"Engelhardt","year":"1990","journal-title":"Arch Neurol"},{"key":"2024032523375850500_nlaa162-B43","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1001\/archneur.1993.00540010026013","article-title":"Lymphocytic infiltrates in the spinal cord in amyotrophic lateral sclerosis","volume":"50","author":"Engelhardt","year":"1993","journal-title":"Arch Neurol"},{"key":"2024032523375850500_nlaa162-B44","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1002\/ana.10805","article-title":"Presence of dendritic cells, MCP-1, and activated microglia\/macrophages in amyotrophic lateral sclerosis spinal cord tissue","volume":"55","author":"Henkel","year":"2004","journal-title":"Ann Neurol"},{"key":"2024032523375850500_nlaa162-B45","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.neulet.2015.05.005","article-title":"Alterations in the blood-spinal cord barrier in TDP-43 conditional knockout mice","volume":"598","author":"Sasaki","year":"2015","journal-title":"Neurosci Lett"},{"key":"2024032523375850500_nlaa162-B46","doi-asserted-by":"crossref","first-page":"4986","DOI":"10.1073\/pnas.1218311110","article-title":"Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth","volume":"110","author":"Schmid","year":"2013","journal-title":"Proc Natl Acad Sci USA"},{"key":"2024032523375850500_nlaa162-B47","first-page":"3419","article-title":"Matrix metalloproteinase-9 is elevated in serum of patients with amyotrophic lateral sclerosis","volume":"11","author":"Beuche","year":"2000","journal-title":"Neuro-Report"},{"key":"2024032523375850500_nlaa162-B48","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.jneuroim.2004.09.015","article-title":"The pro and the active form of matrix metalloproteinase-9 is increased in serum of patients with amyotrophic lateral sclerosis","volume":"159","author":"Demestre","year":"2005","journal-title":"J Neuroimmunol"},{"key":"2024032523375850500_nlaa162-B49","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1111\/j.1468-1331.2009.02775.x","article-title":"Matrix metalloproteinases and their tissue inhibitors in serum and cerebrospinal fluid of patients with amyotrophic lateral sclerosis","volume":"17","author":"Niebroj-Dobosz","year":"2010","journal-title":"Eur J Neurol"},{"key":"2024032523375850500_nlaa162-B50","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1002\/jnr.22594","article-title":"Disruption of neurovascular unit prior to motor neuron degeneration in amyotrophic lateral sclerosis","volume":"89","author":"Miyazaki","year":"2011","journal-title":"J Neurosci Res"},{"key":"2024032523375850500_nlaa162-B51","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1046\/j.1471-4159.1996.67010251.x","article-title":"Matrix metalloproteinases in the neocortex and spinal cord of amyotrophic lateral sclerosis patients","volume":"67","author":"Lim","year":"2002","journal-title":"J Neurochem"},{"key":"2024032523375850500_nlaa162-B52","doi-asserted-by":"crossref","first-page":"546","DOI":"10.3389\/fneur.2017.00546","article-title":"Inflammatory Gene expression in whole peripheral blood at early stages of sporadic amyotrophic lateral sclerosis","volume":"8","author":"Andr\u00e9s-Benito","year":"2017","journal-title":"Front Neurol"},{"key":"2024032523375850500_nlaa162-B53","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1111\/j.1468-1331.2012.03776.x","article-title":"Detection of microbleeds in post-mortem brains of patients with frontotemporal lobar degeneration: A 7.0-Tesla magnetic resonance imaging study with neuropathological correlates","volume":"19","author":"De Reuck","year":"2012","journal-title":"Eur J Neurol"},{"key":"2024032523375850500_nlaa162-B54","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1016\/j.parkreldis.2008.07.005","article-title":"Pallidonigral TDP-43 pathology in Perry syndrome","volume":"15","author":"Wider","year":"2009","journal-title":"Parkinsonism Relat Disord"},{"key":"2024032523375850500_nlaa162-B55","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1097\/NEN.0b013e3181baacec","article-title":"Transactivation response DNA-binding protein 43 microvasculopathy in frontotemporal degeneration and familial Lewy body disease","volume":"68","author":"Lin","year":"2009","journal-title":"J Neuropathol Exp Neurol"},{"key":"2024032523375850500_nlaa162-B56","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1016\/B978-0-12-385157-4.00585-6","volume-title":"Encyclopedia of the Neurological Sciences","author":"Ferrer","year":"2014","edition":"2nd edition"},{"key":"2024032523375850500_nlaa162-B57","doi-asserted-by":"crossref","first-page":"823","DOI":"10.18632\/aging.101195","article-title":"Amyotrophic lateral sclerosis, gene deregulation in the anterior horn of the spinal cord and frontal cortex area 8: Implications in frontotemporal lobar degeneration","volume":"9","author":"Andr\u00e9s-Benito","year":"2017","journal-title":"Aging"},{"key":"2024032523375850500_nlaa162-B58","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1093\/jnen\/nly037","article-title":"Gene expression profile in frontal cortex in sporadic frontotemporal lobar degeneration-TDP","volume":"77","author":"Andr\u00e9s-Benito","year":"2018","journal-title":"Neuropathol Exp Neurol"},{"key":"2024032523375850500_nlaa162-B59","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1007\/s00702-007-0716-6","article-title":"Late-onset frontotemporal dementia associated with a novel PGRN mutation","volume":"114","author":"Llad\u00f3","year":"2007","journal-title":"J Neural Transm"},{"key":"2024032523375850500_nlaa162-B60","doi-asserted-by":"crossref","first-page":"10530","DOI":"10.1523\/JNEUROSCI.3421-07.2007","article-title":"Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43","volume":"27","author":"Zhang","year":"2007","journal-title":"J Neurosci"},{"key":"2024032523375850500_nlaa162-B61","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.brainres.2010.09.099","article-title":"Progranulin deficiency leads to enhanced cell vulnerability and TDP-43 translocation in primary neuronal cultures","volume":"1366","author":"Guo","year":"2010","journal-title":"Brain Res"},{"key":"2024032523375850500_nlaa162-B62","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1007\/s12031-011-9625-0","article-title":"Progranulin and TDP-43: Mechanistic links and future directions","volume":"45","author":"Kumar-Singh","year":"2011","journal-title":"J Mol Neurosci"},{"key":"2024032523375850500_nlaa162-B63","doi-asserted-by":"crossref","first-page":"2611","DOI":"10.1084\/jem.20160999","article-title":"Progranulin deficiency causes impairment of autophagy and TDP-43 accumulation","volume":"214","author":"Chang","year":"2017","journal-title":"Exp Med"},{"key":"2024032523375850500_nlaa162-B64","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/j.mcn.2012.12.006","article-title":"RNA-mediated toxicity in neurodegenerative disease","volume":"56","author":"Belzil","year":"2013","journal-title":"Mol Cell Neurosci"},{"key":"2024032523375850500_nlaa162-B65","doi-asserted-by":"crossref","first-page":"4348","DOI":"10.1111\/febs.12287","article-title":"Stress granules in neurodegeneration\u2013lessons learned from TAR DNA binding protein of 43 kDa and fused in sarcoma","volume":"280","author":"Bentmann","year":"2013","journal-title":"FEBS J"},{"key":"2024032523375850500_nlaa162-B66","doi-asserted-by":"crossref","first-page":"4297","DOI":"10.1016\/j.febslet.2014.09.001","article-title":"tRNA fragments in human health and disease","volume":"588","author":"Anderson","year":"2014","journal-title":"FEBS Lett"},{"key":"2024032523375850500_nlaa162-B67","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":"2024032523375850500_nlaa162-B68","doi-asserted-by":"crossref","first-page":"37572","DOI":"10.1074\/jbc.M505557200","article-title":"TDP-43 binds heterogeneous nuclear ribonucleoprotein A\/B through its C-terminal tail: An important region for the inhibition of cystic fibrosis transmembrane conductance regulator exon 9 splicing","volume":"280","author":"Buratti","year":"2005","journal-title":"J Biol Chem"},{"key":"2024032523375850500_nlaa162-B69","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1111\/j.1471-4159.2007.05190.x","article-title":"TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor","volume":"105","author":"Wang","year":"2008","journal-title":"J Neurochem"},{"key":"2024032523375850500_nlaa162-B70","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1016\/j.brainres.2009.09.105","article-title":"Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins, and copper\/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS)","volume":"1305","author":"Volkening","year":"2009","journal-title":"Brain Res"},{"key":"2024032523375850500_nlaa162-B71","doi-asserted-by":"crossref","first-page":"1774","DOI":"10.1093\/emboj\/20.7.1774","article-title":"Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping","volume":"20","author":"Buratti","year":"2001","journal-title":"EMBO J"},{"key":"2024032523375850500_nlaa162-B72","doi-asserted-by":"crossref","first-page":"R46-6","DOI":"10.1093\/hmg\/ddq137","article-title":"TDP-43 and FUS\/TLS: Emerging roles in RNA processing and neurodegeneration","volume":"19","author":"Lagier-Tourenne","year":"2010","journal-title":"Hum Mol Genet"},{"key":"2024032523375850500_nlaa162-B73","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1038\/nn.2778","article-title":"Characterizing the RNA targets and position-dependent splicing regulation by TDP-43","volume":"14","author":"Tollervey","year":"2011","journal-title":"Nat Neurosci"},{"key":"2024032523375850500_nlaa162-B74","doi-asserted-by":"crossref","first-page":"1661","DOI":"10.1007\/s00702-008-0137-1","article-title":"Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy","volume":"115","author":"Thorpe","year":"2008","journal-title":"J Neural Transm"},{"key":"2024032523375850500_nlaa162-B75","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/j.molmed.2011.06.004","article-title":"TDP-43 functions and pathogenic mechanisms implicated in TDP-43 proteinopathies","volume":"17","author":"Cohen","year":"2011","journal-title":"Trends Mol Med"},{"key":"2024032523375850500_nlaa162-B76","doi-asserted-by":"crossref","first-page":"e13250","DOI":"10.1371\/journal.pone.0013250","article-title":"Tar DNA binding protein-43 (TDP-43) associates with stress granules: Analysis of cultured cells and pathological brain tissue","volume":"5","author":"Liu-Yesucevitz","year":"2010","journal-title":"PLoS One"},{"key":"2024032523375850500_nlaa162-B77","doi-asserted-by":"crossref","first-page":"109591","DOI":"10.1016\/j.cellsig.2020.109591","article-title":"ALS skin fibroblasts reveal oxidative stress and ERK1\/2-mediated cytoplasmic localization of TDP-43","volume":"70","author":"Romano","year":"2020","journal-title":"Cell Signal"},{"key":"2024032523375850500_nlaa162-B78","doi-asserted-by":"crossref","first-page":"105051","DOI":"10.1016\/j.nbd.2020.105051","article-title":"Chronic stress induces formation of stress granules and pathological TDP-43 aggregates in human ALS fibroblasts and iPSC-motoneurons","volume":"145","author":"Ratti","year":"2020","journal-title":"Neurobiol Dis"},{"key":"2024032523375850500_nlaa162-B79","doi-asserted-by":"crossref","first-page":"1291","DOI":"10.1007\/s00415-020-09704-8","article-title":"ALS-derived fibroblasts exhibit reduced proliferation rate, cytoplasmic TDP-43 aggregation, and a higher susceptibility to DNA damage","volume":"267","author":"Riancho","year":"2020","journal-title":"J Neurol"},{"key":"2024032523375850500_nlaa162-B80","doi-asserted-by":"crossref","first-page":"3883","DOI":"10.1093\/hmg\/ddt243","article-title":"Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLD","volume":"22","author":"Diaper","year":"2013","journal-title":"Hum Mol Genet"},{"key":"2024032523375850500_nlaa162-B81","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1136\/jnnp.2007.131334","article-title":"TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia","volume":"79","author":"Weihl","year":"2008","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2024032523375850500_nlaa162-B82","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1097\/NEN.0b013e3181996d8f","article-title":"TAR DNA-Binding protein 43 accumulation in protein aggregate myopathies","volume":"68","author":"Oliv\u00e9","year":"2009","journal-title":"J Neuropathol Exp Neurol"},{"key":"2024032523375850500_nlaa162-B83","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1007\/s12031-011-9627-y","article-title":"The multiple faces of valosin-containing protein-associated diseases: Inclusion body myopathy with Paget\u2019s disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis","volume":"45","author":"Nalbandian","year":"2011","journal-title":"J Mol Neurosci"}],"container-title":["Journal of Neuropathology &amp; Experimental Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jnen\/article-pdf\/80\/3\/229\/36335506\/nlaa162.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jnen\/article-pdf\/80\/3\/229\/36335506\/nlaa162.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,26]],"date-time":"2024-03-26T00:00:36Z","timestamp":1711411236000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jnen\/article\/80\/3\/229\/6075095"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,9]]},"references-count":83,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2021,1,9]]},"published-print":{"date-parts":[[2021,2,22]]}},"URL":"https:\/\/doi.org\/10.1093\/jnen\/nlaa162","relation":{},"ISSN":["0022-3069","1554-6578"],"issn-type":[{"value":"0022-3069","type":"print"},{"value":"1554-6578","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,3,1]]},"published":{"date-parts":[[2021,1,9]]}}}