{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T22:23:36Z","timestamp":1790375016587,"version":"4.1.0"},"reference-count":17,"publisher":"Wiley","issue":"17","license":[{"start":{"date-parts":[[2020,7,15]],"date-time":"2020-07-15T00:00:00Z","timestamp":1594771200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002261","name":"Russian Foundation for Basic Research","doi-asserted-by":"publisher","award":["RFBR\u201018\u2010015\u201000450"],"award-info":[{"award-number":["RFBR\u201018\u2010015\u201000450"]}],"id":[{"id":"10.13039\/501100002261","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006769","name":"Russian Science Foundation","doi-asserted-by":"publisher","award":["RSF\u201018\u201015\u201000357"],"award-info":[{"award-number":["RSF\u201018\u201015\u201000357"]}],"id":[{"id":"10.13039\/501100006769","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cellular Molecular Medi"],"published-print":{"date-parts":[[2020,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Genetic mutations in FUS, a DNA\/RNA\u2010binding protein, are associated with inherited forms of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). A novel transgenic FUS[1\u2010359]\u2010tg mouse line recapitulates core hallmarks of human ALS in the spinal cord, including neuroinflammation and neurodegeneration, ensuing muscle atrophy and paralysis, as well as brain pathomorphological signs of FTLD. However, a question whether FUS[1\u2010359]\u2010tg mouse displays behavioural and brain pro\u2010inflammatory changes characteristic for the FTLD syndrome was not addressed. Here, we studied emotional, social and cognitive behaviours, brain markers of inflammation and plasticity of pre\u2010symptomatic FUS[1\u2010359]\u2010tg male mice, a potential FTLD model. These animals displayed aberrant behaviours and altered brain expression of inflammatory markers and related pathways that are reminiscent to the FTLD\u2010like syndrome. FTLD\u2010related behavioural and molecular Journal of Cellular and Molecular Medicine features were studied in the pre\u2010symptomatic FUS[1\u2010359]\u2010tg mice that received standard or new ALS treatments, which have been reported to counteract the ALS\u2010like syndrome in the mutants. We used anti\u2010ALS drug riluzole (8\u00a0mg\/kg\/d), or anti\u2010inflammatory drug, a selective blocker of cyclooxygenase\u20102 (celecoxib, 30\u00a0mg\/kg\/d) for 3\u00a0weeks, or a single intracerebroventricular (i.c.v.) infusion of human stem cells (Neuro\u2010Cells, 500\u00a0000\u2010CD34<jats:sup>+<\/jats:sup>), which showed anti\u2010inflammatory properties. Signs of elevated anxiety, depressive\u2010like behaviour, cognitive deficits and abnormal social behaviour were less marked in FUS\u2010tg\u2013treated animals. Applied treatments have normalized protein expression of interleukin\u20101\u03b2 (IL\u20101\u03b2) in the prefrontal cortex and the hippocampus, and of Iba\u20101 and GSK\u20103\u03b2 in the hippocampus. Thus, the pre\u2010symptomatic FUS[1\u2010359]\u2010tg mice demonstrate FTLD\u2010like abnormalities that are attenuated by standard and new ALS treatments, including Neuro\u2010Cell preparation.<\/jats:p>","DOI":"10.1111\/jcmm.15628","type":"journal-article","created":{"date-parts":[[2020,7,15]],"date-time":"2020-07-15T12:07:35Z","timestamp":1594814855000},"page":"10251-10257","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Molecular and behavioural abnormalities in the FUS\u2010tg mice mimic frontotemporal lobar degeneration: Effects of old and new anti\u2010inflammatory therapies"],"prefix":"10.1111","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7583-1464","authenticated-orcid":false,"given":"Johannes","family":"de Munter","sequence":"first","affiliation":[{"name":"Department of Psychiatry and Neuropsychology School for Mental Health and Neuroscience Maastricht University Maastricht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1692-0445","authenticated-orcid":false,"given":"Diana","family":"Babaevskaya","sequence":"additional","affiliation":[{"name":"Laboratory of Psychiatric Neurobiology, Institute of Molecular Medicine and Department of Normal Physiology I.M. Sechenov First Moscow State Medical University Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4411-6892","authenticated-orcid":false,"given":"Erik Ch.","family":"Wolters","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Neuropsychology School for Mental Health and Neuroscience Maastricht University Maastricht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9120-7266","authenticated-orcid":false,"given":"Dmitrii","family":"Pavlov","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Neuropsychology School for Mental Health and Neuroscience Maastricht University Maastricht The Netherlands"},{"name":"Laboratory of Psychiatric Neurobiology, Institute of Molecular Medicine and Department of Normal Physiology I.M. Sechenov First Moscow State Medical University Moscow Russia"},{"name":"Laboratory of Cognitive Dysfunctions Institute of General Pathology and Pathophysiology Moscow Russia"},{"name":"Department of Advanced Cell Technologies, Institute of Regenerative Medicine I.M. Sechenov First Moscow State Medical University Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6521-3793","authenticated-orcid":false,"given":"Ekaterina","family":"Lysikova","sequence":"additional","affiliation":[{"name":"Institute of Physiologically Active Compounds Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7525-1950","authenticated-orcid":false,"given":"Allan","family":"V. Kalueff","sequence":"additional","affiliation":[{"name":"Faculty of Biology Ural Federal University Ekaterinburg Russia"},{"name":"Institute of Translational Biomedicine and Institute of Experimental Medicine St. Petersburg State University and Almazov Medical Research Center St. Petersburg Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9345-7880","authenticated-orcid":false,"given":"Anna","family":"Gorlova","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Neuropsychology School for Mental Health and Neuroscience Maastricht University Maastricht The Netherlands"},{"name":"Laboratory of Psychiatric Neurobiology, Institute of Molecular Medicine and Department of Normal Physiology I.M. Sechenov First Moscow State Medical University Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7641-2330","authenticated-orcid":false,"given":"Margarita","family":"Oplatchikova","sequence":"additional","affiliation":[{"name":"Laboratory of Psychiatric Neurobiology, Institute of Molecular Medicine and Department of Normal Physiology I.M. Sechenov First Moscow State Medical University Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8426-3371","authenticated-orcid":false,"given":"Igor A.","family":"Pomytkin","sequence":"additional","affiliation":[{"name":"Department of Advanced Cell Technologies I.M. Sechenov First Moscow State Medical University Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7258-7870","authenticated-orcid":false,"given":"Andrey","family":"Proshin","sequence":"additional","affiliation":[{"name":"PK Anokhin Research Institute of Normal Physiology Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1637-4245","authenticated-orcid":false,"given":"Aleksei","family":"Umriukhin","sequence":"additional","affiliation":[{"name":"Laboratory of Psychiatric Neurobiology, Institute of Molecular Medicine and Department of Normal Physiology I.M. Sechenov First Moscow State Medical University Moscow Russia"},{"name":"PK Anokhin Research Institute of Normal Physiology Moscow Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8348-153X","authenticated-orcid":false,"given":"Klaus\u2010Peter","family":"Lesch","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Neuropsychology School for Mental Health and Neuroscience Maastricht University Maastricht The Netherlands"},{"name":"Laboratory of Psychiatric Neurobiology, Institute of Molecular Medicine and Department of Normal Physiology I.M. Sechenov First Moscow State Medical University Moscow Russia"},{"name":"Division of Molecular Psychiatry Center of Mental Health University of W\u00fcrzburg W\u00fcrzburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9937-5600","authenticated-orcid":false,"given":"Tatyana","family":"Strekalova","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Neuropsychology School for Mental Health and Neuroscience Maastricht University Maastricht The Netherlands"},{"name":"Laboratory of Psychiatric Neurobiology, Institute of Molecular Medicine and Department of Normal Physiology I.M. Sechenov First Moscow State Medical University Moscow Russia"},{"name":"Division of Molecular Psychiatry Center of Mental Health University of W\u00fcrzburg W\u00fcrzburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,7,15]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3182343365"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1517\/17460441.2015.1067197"},{"key":"e_1_2_9_4_1","first-page":"102","article-title":"Dendritic homeostasis disruption in a novel frontotemporal dementia mouse model expressing cytoplasmic fused in sarcoma","volume":"24","author":"Shiihashi G","year":"2017","journal-title":"E\u2010Biological Medicine"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12868-016-0304-5"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40478-016-0358-8"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.492017"},{"key":"e_1_2_9_8_1","doi-asserted-by":"crossref","first-page":"12607","DOI":"10.1111\/gbb.12607","article-title":"Behavioural impairments in mice of a novel FUS transgenic line recapitulate features of frontotemporal lobar degeneration","volume":"22","author":"Lysikova EA","year":"2019","journal-title":"Genes Brain Behavior"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/cns.13280"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pnpbp.2009.12.014"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2019.05.002"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12974-016-0572-0"},{"key":"e_1_2_9_13_1","first-page":"4639","article-title":"Behavioral deficits and progressive neuropathology in progranulin\u2010deficient mice: a mouse model of frontotemporal dementia","volume":"12","author":"Yin F","year":"2010","journal-title":"FASEB J"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnagi.2016.00310"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.6103-11.2013"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-199909090-00018"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.21273"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12640-017-9720-2"}],"container-title":["Journal of Cellular and Molecular Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjcmm.15628","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jcmm.15628","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/jcmm.15628","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jcmm.15628","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,4]],"date-time":"2023-10-04T08:00:15Z","timestamp":1696406415000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jcmm.15628"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,7,15]]},"references-count":17,"journal-issue":{"issue":"17","published-print":{"date-parts":[[2020,9]]}},"alternative-id":["10.1111\/jcmm.15628"],"URL":"https:\/\/doi.org\/10.1111\/jcmm.15628","archive":["Portico"],"relation":{},"ISSN":["1582-1838","1582-4934"],"issn-type":[{"value":"1582-1838","type":"print"},{"value":"1582-4934","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,7,15]]},"assertion":[{"value":"2020-04-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-06-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-07-15","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}