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Williams syndrome is caused by a microdeletion of 26\u201328 genes on chromosome 7, including <jats:italic>GTF2IRD1<\/jats:italic>, which encodes a transcription factor suggested to play a role in the behavioral profile of Williams syndrome. Duplications of the full region also lead to frequent autism diagnosis, social phobias and language delay. Thus, genes in the region appear to regulate social motivation in a dose\u2010sensitive manner. A \u201ccomplete deletion\u201d mouse, heterozygously eliminating the syntenic Williams syndrome region, has been deeply characterized for cardiac phenotypes, but direct measures of social motivation have not been assessed. Furthermore, the role of <jats:italic>Gtf2ird1<\/jats:italic> in these behaviors has not been addressed in a relevant genetic context. Here, we have generated a mouse overexpressing <jats:italic>Gtf2ird1<\/jats:italic>, which can be used both to model duplication of this gene alone and to rescue <jats:italic>Gtf2ird1<\/jats:italic> expression in the complete deletion mice. Using a comprehensive behavioral pipeline and direct measures of social motivation, we provide evidence that the Williams syndrome critical region regulates social motivation along with motor and anxiety phenotypes, but that <jats:italic>Gtf2ird1<\/jats:italic> complementation is not sufficient to rescue most of these traits, and duplication does not decrease social motivation. However, <jats:italic>Gtf2ird1<\/jats:italic> complementation does rescue light\u2010aversive behavior and performance on select sensorimotor tasks, perhaps indicating a role for this gene in sensory processing or integration.<\/jats:p>","DOI":"10.1111\/gbb.12853","type":"journal-article","created":{"date-parts":[[2023,6,28]],"date-time":"2023-06-28T04:44:06Z","timestamp":1687927446000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Extensive characterization of a Williams syndrome murine model shows <i>Gtf2ird1<\/i>\u2010mediated rescue of select sensorimotor tasks, but no effect on enhanced social behavior"],"prefix":"10.1111","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1640-3026","authenticated-orcid":false,"given":"Kayla R.","family":"Nygaard","sequence":"first","affiliation":[{"name":"Department of Genetics Washington University School of Medicine  St. Louis Missouri USA"},{"name":"Department of Psychiatry Washington University School of Medicine  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5585-4722","authenticated-orcid":false,"given":"Susan E.","family":"Maloney","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Washington University School of Medicine  St. Louis Missouri USA"},{"name":"Intellectual &amp; Developmental Disabilities Research Center Washington University School of Medicine  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raylynn G.","family":"Swift","sequence":"additional","affiliation":[{"name":"Department of Genetics Washington University School of Medicine  St. Louis Missouri USA"},{"name":"Department of Psychiatry Washington University School of Medicine  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine B.","family":"McCullough","sequence":"additional","affiliation":[{"name":"Department of Genetics Washington University School of Medicine  St. Louis Missouri USA"},{"name":"Department of Psychiatry Washington University School of Medicine  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachael E.","family":"Wagner","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Washington University School of Medicine  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart B.","family":"Fass","sequence":"additional","affiliation":[{"name":"Department of Genetics Washington University School of Medicine  St. Louis Missouri USA"},{"name":"Department of Psychiatry Washington University School of Medicine  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krassimira","family":"Garbett","sequence":"additional","affiliation":[{"name":"Department of Pharmacology Vanderbilt University  Nashville Tennessee USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karoly","family":"Mirnics","sequence":"additional","affiliation":[{"name":"Psychiatry, Biochemistry &amp; Molecular Biology, Pharmacology &amp; Experimental Neuroscience, Munroe\u2010Meyer Institute for Genetics and Rehabilitation University of Nebraska Medical Center  Omaha Nebraska USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy","family":"Veenstra\u2010VanderWeele","sequence":"additional","affiliation":[{"name":"Departments of Psychiatry and Pediatrics Columbia University, New York State Psychiatric Institute, and Center for Autism and the Developing Brain, New York\u2010Presbyterian Hospital  New York City New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6385-3997","authenticated-orcid":false,"given":"Joseph 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