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Individuals with Fragile X syndrome (FXS), the most common heritable form of intellectual disability, have a full-mutation sequence (&gt;200 CGG repeats) which brings about transcriptional silencing of FMR1 and loss of FMR protein (FMRP). Despite considerable progress in our understanding of FXS, safe, effective, and reliable treatments that either prevent or reduce the severity of the FXS phenotype have not been approved. While current FXS animal models contribute their own unique understanding to the molecular, cellular, physiological, and behavioral deficits associated with FXS, no single animal model is able to fully recreate the FXS phenotype. This review will describe the status and rationale in the development, validation, and utility of three emerging animal model systems for FXS, namely the nonhuman primate (NHP), Mongolian gerbil, and chicken. These developing animal models will provide a sophisticated resource in which the deficits in complex functions of perception, action, and cognition in the human disorder are accurately reflected and aid in the successful translation of novel therapeutics and interventions to the clinic setting.<\/jats:p>","DOI":"10.3390\/cells11101628","type":"journal-article","created":{"date-parts":[[2022,5,12]],"date-time":"2022-05-12T21:46:53Z","timestamp":1652392013000},"page":"1628","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["New Animal Models for Understanding FMRP Functions and FXS Pathology"],"prefix":"10.3390","volume":"11","author":[{"given":"Eliza","family":"Curnow","sequence":"first","affiliation":[{"name":"REI Division, Department of ObGyn, University of Washington, Seattle, WA 98195, USA"},{"name":"Washington National Primate Research Center, University of Washington, Seattle, WA 98195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuan","family":"Wang","sequence":"additional","affiliation":[{"name":"Program in Neuroscience, Department of Biomedical Sciences, College of Medicine, Florida State University, Tallahassee, FL 32306, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1016\/0092-8674(91)90125-I","article-title":"Absence of Expression of the FMR-1 Gene in Fragile X Syndrome","volume":"66","author":"Pieretti","year":"1991","journal-title":"Cell"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1038\/363722a0","article-title":"Characterization and Localization of the FMR-1 Gene Product Associated with Fragile X Syndrome","volume":"363","author":"Verheij","year":"1993","journal-title":"Nature"},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Zhao, X., and Usdin, K. 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