{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:46:05Z","timestamp":1792788365535,"version":"4.5.0"},"reference-count":72,"publisher":"The Company of Biologists","issue":"9","license":[{"start":{"date-parts":[[2021,9,21]],"date-time":"2021-09-21T00:00:00Z","timestamp":1632182400000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100004794","name":"Centre National de la Recherche Scientifique","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004794","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100007241","name":"Universit\u00e9 Paris-Saclay","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100007241","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Association Mon\u00e9gasque contre les Myopathies"},{"DOI":"10.13039\/501100019973","name":"Minist\u00e8re de l'Enseignement Sup\u00e9rieur et de la Recherche","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100019973","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100010661","name":"Horizon 2020 Framework Programme","doi-asserted-by":"crossref","award":["847826"],"award-info":[{"award-number":["847826"]}],"id":[{"id":"10.13039\/100010661","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,9,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>The exon-52-deleted mdx52 mouse is a critical model of Duchenne muscular dystrophy (DMD), as it features a deletion in a hotspot region of the DMD gene, frequently mutated in patients. Deletion of exon 52 impedes expression of several brain dystrophins (Dp427, Dp260 and Dp140), thus providing a key model for studying the cognitive impairment associated with DMD and testing rescuing strategies. Here, using in vivo magnetic resonance imaging and neurohistology, we found no gross brain abnormalities in mdx52 mice, suggesting that the neural dysfunctions in this model are likely at the level of brain cellular functionalities. Then, we investigated emotional behavior and fear learning performance of mdx52 mice compared to mdx mice that only lack Dp427 to focus on behavioral phenotypes that could be used in future comparative preclinical studies. mdx52 mice displayed enhanced anxiety and a severe impairment in learning an amygdala-dependent Pavlovian association. These replicable behavioral outcome measures are reminiscent of the internalizing problems reported in a quarter of DMD patients, and will be useful for preclinical estimation of the efficacy of treatments targeting brain dysfunctions in DMD.<\/jats:p>","DOI":"10.1242\/dmm.049028","type":"journal-article","created":{"date-parts":[[2023,5,25]],"date-time":"2023-05-25T03:33:44Z","timestamp":1684985624000},"source":"Crossref","is-referenced-by-count":39,"title":["Emotional behavior and brain anatomy of the\n                    <i>mdx52<\/i>\n                    mouse model of Duchenne muscular dystrophy"],"prefix":"10.1242","volume":"14","author":[{"given":"Amel","family":"Saoudi","sequence":"first","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay 1 , 91190, Gif-sur-Yvette , France"},{"name":"Universit\u00e9 Paris-Saclay, UVSQ, Inserm, END-ICAP 2 , 78000 Versailles , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faouzi","family":"Zarrouki","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay 1 , 91190, Gif-sur-Yvette , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Sebri\u00e9","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, CEA, CNRS, Inserm, BioMaps, Service Hospitalier Fr\u00e9d\u00e9ric Joliot, 4 place du g\u00e9n\u00e9ral Leclerc 3 , 91401 Orsay , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5723-3129","authenticated-orcid":false,"given":"Charlotte","family":"Izabelle","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay 1 , 91190, Gif-sur-Yvette , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aur\u00e9lie","family":"Goyenvalle","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, UVSQ, Inserm, END-ICAP 2 , 78000 Versailles , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8783-8185","authenticated-orcid":false,"given":"Cyrille","family":"Vaillend","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, CNRS, Institut des Neurosciences Paris Saclay 1 , 91190, Gif-sur-Yvette , France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2021,9,21]]},"reference":[{"key":"2023052507332754600_DMM049028C1","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1093\/brain\/awf012","article-title":"Brain function in Duchenne muscular dystrophy","volume":"125","author":"Anderson","year":"2002","journal-title":"Brain J. 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