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Pathological inclusions of fused in sarcoma (FUS) protein have been observed in subsets of patients with these diseases but their anatomical distribution is different for two diseases. These structures are present in motor neurons in ALS cases but in cortical neurons in FTLD cases. Expression of a C\u2010terminally truncated form of human FUS causes an early onset and progressive motor neuron pathology in transgenic mice but only when these neurons express a certain level of this protein. Severe motor dysfunction and early lethality of mice with expression above this level prevent their use for studies of FTLD\u2010related pathology caused by expression of this form of FUS. In the present study, we used another line of mice expressing the same protein but not developing any signs of motor system dysfunction due to substantially lower level of transgene expression in motor neurons. In a set of tests 5\u2010month old mice displayed certain behavioural abnormalities, including increased impulsivity, decreased anxiety and compromised social interaction, which recapitulate behaviour characteristics typically seen in FTLD patients.<\/jats:p>","DOI":"10.1111\/gbb.12607","type":"journal-article","created":{"date-parts":[[2019,8,22]],"date-time":"2019-08-22T16:28:17Z","timestamp":1566491297000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Behavioural impairments in mice of a novel FUS transgenic line recapitulate features of frontotemporal lobar degeneration"],"prefix":"10.1111","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6521-3793","authenticated-orcid":false,"given":"Ekaterina A.","family":"Lysikova","sequence":"first","affiliation":[{"name":"School of Biosciences Cardiff University  Cardiff UK"},{"name":"Institute of Physiologically Active Compounds, Russian Academy of Sciences  Chernogolovka Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michail S.","family":"Kukharsky","sequence":"additional","affiliation":[{"name":"Institute of Physiologically Active Compounds, Russian Academy of Sciences  Chernogolovka Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kirill D.","family":"Chaprov","sequence":"additional","affiliation":[{"name":"School of Biosciences Cardiff University  Cardiff UK"},{"name":"Institute of Physiologically Active Compounds, Russian Academy of Sciences  Chernogolovka Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nataliia A.","family":"Vasilieva","sequence":"additional","affiliation":[{"name":"Institute of Physiologically Active Compounds, Russian Academy of Sciences  Chernogolovka Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrei Y.","family":"Roman","sequence":"additional","affiliation":[{"name":"Institute of Physiologically Active Compounds, Russian Academy of Sciences  Chernogolovka Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruslan K.","family":"Ovchinnikov","sequence":"additional","affiliation":[{"name":"Institute of Physiologically Active Compounds, Russian Academy of Sciences  Chernogolovka Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexey V.","family":"Deykin","sequence":"additional","affiliation":[{"name":"Institute of Gene Biology, Russian Academy of Sciences  Moscow Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"Ninkina","sequence":"additional","affiliation":[{"name":"School of Biosciences Cardiff University  Cardiff UK"},{"name":"Institute of Physiologically Active Compounds, Russian Academy of Sciences  Chernogolovka Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vladimir L.","family":"Buchman","sequence":"additional","affiliation":[{"name":"School of Biosciences Cardiff University  Cardiff UK"},{"name":"Institute of Physiologically Active Compounds, Russian Academy of Sciences  Chernogolovka Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,9,10]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/nan.12526"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.13654"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.15252\/embr.201845766"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1080\/15476286.2016.1211225"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181ccc732"},{"issue":"3","key":"e_1_2_7_7_1","first-page":"765","article-title":"FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosis","volume":"22","author":"Broustal O","year":"2010","journal-title":"J Alzheimers Dis"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2009.194399"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1002011"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.492017"},{"key":"e_1_2_7_11_1","doi-asserted-by":"crossref","unstructured":"LysikovaEA FunikovS RezvykhAP et al. 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