{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T02:56:51Z","timestamp":1785293811660,"version":"3.55.0"},"reference-count":68,"publisher":"The Company of Biologists","issue":"8","license":[{"start":{"date-parts":[[2025,8,26]],"date-time":"2025-08-26T00:00:00Z","timestamp":1756166400000},"content-version":"vor","delay-in-days":25,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100017510","name":"UK Dementia Research Institute","doi-asserted-by":"crossref","award":["UKDRI-1014"],"award-info":[{"award-number":["UKDRI-1014"]}],"id":[{"id":"10.13039\/501100017510","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100017510","name":"UK Dementia Research Institute","doi-asserted-by":"crossref","award":["UKDRI-CIP0202"],"award-info":[{"award-number":["UKDRI-CIP0202"]}],"id":[{"id":"10.13039\/501100017510","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100017510","name":"UK Dementia Research Institute","doi-asserted-by":"crossref","award":["UKDRI-1203"],"award-info":[{"award-number":["UKDRI-1203"]}],"id":[{"id":"10.13039\/501100017510","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Alzheimer's Research UK","award":["ARUK-SRF2018A-001"],"award-info":[{"award-number":["ARUK-SRF2018A-001"]}]},{"DOI":"10.13039\/501100000406","name":"Motor Neurone Disease Association","doi-asserted-by":"crossref","award":["Isaacs\/Apr20\/876-791"],"award-info":[{"award-number":["Isaacs\/Apr20\/876-791"]}],"id":[{"id":"10.13039\/501100000406","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Medical Research Council","award":["A410-53658"],"award-info":[{"award-number":["A410-53658"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,8,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are progressive adult-onset neurodegenerative diseases with overlapping pathological and genetic origins. They are caused by multiple underlying mechanisms leading to a common collection of clinical features that occur in a spectrum. Here, we report side-by-side longitudinal behavioural, cognitive and sensory phenotyping of two mouse models of ALS\/FTD, to determine which aspects of the disease they recapitulate. We used knock-in models, in which the endogenous mouse orthologues of the C9orf72 and TARDBP (encoding TDP-43) genes have been altered to model specific molecular aspects of ALS\/FTD. We found that the C9orf72GR400\/+ model exhibits age-related deficit in short-term memory and that parental genotype affects exploration activity in offspring. In the TardbpQ331K\/Q331K model, we found age-related changes in weight, fat mass, locomotion and marble burying. In both models, we found no evidence of deficits in vision or olfactory habituation-dishabituation. These data provide new insight into genotype-phenotype relationships in these ALS\/FTD mice, which can be used to inform model choice and experimental design in future research studies.<\/jats:p>","DOI":"10.1242\/dmm.052324","type":"journal-article","created":{"date-parts":[[2025,8,26]],"date-time":"2025-08-26T07:52:47Z","timestamp":1756194767000},"source":"Crossref","is-referenced-by-count":6,"title":["Multi-modal comparative phenotyping of knock-in mouse models of frontotemporal dementia\/amyotrophic lateral sclerosis"],"prefix":"10.1242","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3321-3160","authenticated-orcid":false,"given":"Sevda","family":"Boyanova","sequence":"first","affiliation":[{"name":"UK Dementia Research Institute, University College London 1 , London WC1E 6BT ,","place":["UK"]},{"name":"UCL Queen Square Institute of Neurology, University College London 2 , London WC1N 3BG ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8647-667X","authenticated-orcid":false,"given":"Gareth","family":"Banks","sequence":"additional","affiliation":[{"name":"UK Dementia Research Institute, University College London 1 , London WC1E 6BT ,","place":["UK"]},{"name":"Nottingham Trent University 3 School of Science and Technology , , Clifton Lane, Nottingham NG11 8NF ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1823-465X","authenticated-orcid":false,"given":"Tatiana V.","family":"Lipina","sequence":"additional","affiliation":[{"name":"UK Dementia Research Institute, University College London 1 , London WC1E 6BT ,","place":["UK"]},{"name":"University of Toronto 4 , Department of Pharmacology and Toxicology, Toronto Ontario M5G 2C8 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0443-7704","authenticated-orcid":false,"given":"Rasneer Sonia","family":"Bains","sequence":"additional","affiliation":[{"name":"Mary Lyon Centre, Medical Research Council Harwell 5 , Oxfordshire OX11 0RD ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-3120-8054","authenticated-orcid":false,"given":"Hamish","family":"Forrest","sequence":"additional","affiliation":[{"name":"Mary Lyon Centre, Medical Research Council Harwell 5 , Oxfordshire OX11 0RD ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6787-5288","authenticated-orcid":false,"given":"Michelle","family":"Stewart","sequence":"additional","affiliation":[{"name":"Mary Lyon Centre, Medical Research Council Harwell 5 , Oxfordshire OX11 0RD ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mireia","family":"Carcol\u00e9","sequence":"additional","affiliation":[{"name":"UK Dementia Research Institute, University College London 1 , London WC1E 6BT ,","place":["UK"]},{"name":"UCL Queen Square Institute of Neurology, University College London 2 , London WC1N 3BG ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmelo","family":"Milioto","sequence":"additional","affiliation":[{"name":"UK Dementia Research Institute, University College London 1 , London WC1E 6BT ,","place":["UK"]},{"name":"UCL Queen Square Institute of Neurology, University College London 2 , London WC1N 3BG ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6820-5534","authenticated-orcid":false,"given":"Adrian M.","family":"Isaacs","sequence":"additional","affiliation":[{"name":"UK Dementia Research Institute, University College London 1 , London WC1E 6BT ,","place":["UK"]},{"name":"UCL Queen Square Institute of Neurology, University College London 2 , London WC1N 3BG ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0572-0600","authenticated-orcid":false,"given":"Sara E.","family":"Wells","sequence":"additional","affiliation":[{"name":"Mary Lyon Centre, Medical Research Council Harwell 5 , Oxfordshire OX11 0RD ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9323-3548","authenticated-orcid":false,"given":"Frances K.","family":"Wiseman","sequence":"additional","affiliation":[{"name":"UK Dementia Research Institute, University College London 1 , London WC1E 6BT ,","place":["UK"]},{"name":"UCL Queen Square Institute of Neurology, University College London 2 , London WC1N 3BG 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