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The understanding of genotype\u2013phenotype relationships, the identification of driver genes and various proofs of concept for therapeutics have benefited from mouse models. The premier model, named Ts(1716)65Dn\/J (Ts65Dn), displayed phenotypes related to human DS features. It carries an additional minichromosome with the Mir155 to Zbtb21 region of mouse chromosome 16, homologous to Hsa21, encompassing around 90 genes, fused to the centromeric part of mouse chromosome 17 from Pisd-ps2\/Scaf8 to Pde10a, containing 46 genes not related to Hsa21. Here, we report the investigation of a new model, Ts66Yah, generated by CRISPR\/Cas9 without the genomic region unrelated to Hsa21 on the minichromosome. As expected, Ts66Yah replicated DS cognitive features. However, certain phenotypes related to increased activity, spatial learning and molecular signatures were changed, suggesting genetic interactions between the Mir155-Zbtb21 and Scaf8-Pde10a intervals. Thus, Ts66Yah mice have stronger construct and face validity than Ts65Dn mice for mimicking consequences of DS genetic overdosage. Furthermore, this study is the first to demonstrate genetic interactions between triplicated regions homologous to Hsa21 and others unrelated to Hsa21.<\/jats:p>\n                  <jats:p>This article has an associated First Person interview with the first author of the paper.<\/jats:p>","DOI":"10.1242\/dmm.049721","type":"journal-article","created":{"date-parts":[[2022,11,14]],"date-time":"2022-11-14T09:29:43Z","timestamp":1668418183000},"source":"Crossref","is-referenced-by-count":61,"title":["Ts66Yah, a mouse model of Down syndrome with improved construct and face validity"],"prefix":"10.1242","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3043-702X","authenticated-orcid":false,"given":"Arnaud","family":"Duchon","sequence":"first","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS, INSERM, Institut de G\u00e9n\u00e9tique et de Biologie Mol\u00e9culaire et Cellulaire (IGBMC) 1 , Department of Translational Medicine and Neurogenetics, 1 rue Laurent Fries, 67404 Illkirch-Graffenstaden , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"del Mar Mu\u00f1iz Moreno","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS, INSERM, Institut de G\u00e9n\u00e9tique et de Biologie Mol\u00e9culaire et Cellulaire (IGBMC) 1 , Department of Translational Medicine and Neurogenetics, 1 rue Laurent Fries, 67404 Illkirch-Graffenstaden , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Chevalier","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS, INSERM, Institut de G\u00e9n\u00e9tique et de Biologie Mol\u00e9culaire et Cellulaire (IGBMC) 1 , Department of Translational Medicine and Neurogenetics, 1 rue Laurent Fries, 67404 Illkirch-Graffenstaden , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6089-2504","authenticated-orcid":false,"given":"Val\u00e9rie","family":"Nalesso","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS, INSERM, Institut de G\u00e9n\u00e9tique et de Biologie Mol\u00e9culaire et Cellulaire (IGBMC) 1 , Department of Translational Medicine and Neurogenetics, 1 rue Laurent Fries, 67404 Illkirch-Graffenstaden , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Andre","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS, INSERM, CELPHEDIA, PHENOMIN-Institut Clinique de la Souris (ICS) 2 , 1 rue Laurent Fries, 67404 Illkirch-Graffenstaden , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2345-385X","authenticated-orcid":false,"given":"Marta","family":"Fructuoso-Castellar","sequence":"additional","affiliation":[{"name":"Paris Brain Institute ICM, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re 3 , 75013 Paris , France"},{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U1127, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re 4 , 75013 Paris , France"},{"name":"Centre National de la Recherche Scientifique, UMR 7225, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re 5 , 75013 Paris , France"},{"name":"Sorbonne Universit\u00e9, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re 6 , 75013 Paris , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1770-7435","authenticated-orcid":false,"given":"Mary","family":"Mondino","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS UMR 7357, ICube, FMTS 7 , 67000 Strasbourg , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9785-9572","authenticated-orcid":false,"given":"Chrystelle","family":"Po","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS UMR 7357, ICube, FMTS 7 , 67000 Strasbourg , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3655-3163","authenticated-orcid":false,"given":"Vincent","family":"Noblet","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS UMR 7357, ICube, FMTS 7 , 67000 Strasbourg , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3372-8108","authenticated-orcid":false,"given":"Marie-Christine","family":"Birling","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS, INSERM, CELPHEDIA, PHENOMIN-Institut Clinique de la Souris (ICS) 2 , 1 rue Laurent Fries, 67404 Illkirch-Graffenstaden , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2462-7150","authenticated-orcid":false,"given":"Marie-Claude","family":"Potier","sequence":"additional","affiliation":[{"name":"Paris Brain Institute ICM, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re 3 , 75013 Paris , France"},{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, U1127, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re 4 , 75013 Paris , France"},{"name":"Centre National de la Recherche Scientifique, UMR 7225, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re 5 , 75013 Paris , France"},{"name":"Sorbonne Universit\u00e9, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re 6 , 75013 Paris , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7049-6900","authenticated-orcid":false,"given":"Yann","family":"Herault","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, CNRS, INSERM, Institut de G\u00e9n\u00e9tique et de Biologie Mol\u00e9culaire et Cellulaire (IGBMC) 1 , Department of Translational Medicine and Neurogenetics, 1 rue Laurent Fries, 67404 Illkirch-Graffenstaden , France"},{"name":"Universit\u00e9 de Strasbourg, CNRS, INSERM, CELPHEDIA, PHENOMIN-Institut Clinique de la Souris (ICS) 2 , 1 rue Laurent Fries, 67404 Illkirch-Graffenstaden , France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2022,12,6]]},"reference":[{"key":"2022120614191411200_DMM049721C53","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1111\/2041-210X.12035","article-title":"geomorph: an r package for the collection and analysis of geometric 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Mech."},{"key":"2022120614191411200_DMM049721C61","doi-asserted-by":"publisher","first-page":"1306","DOI":"10.1038\/nprot.2006.205","article-title":"Object recognition in rats and mice: a one-trial non-matching-to-sample learning task to study 'recognition memory","volume":"1","author":"Bevins","year":"2006","journal-title":"Nat. Protoc."},{"key":"2022120614191411200_DMM049721C57","doi-asserted-by":"publisher","first-page":"43331","DOI":"10.1038\/srep43331","article-title":"Efficient and rapid generation of large genomic variants in rats and mice using CRISMERE","volume":"7","author":"Birling","year":"2017","journal-title":"Sci. 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