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Prog Brain Res. 2012;197:169-97. doi: 10.1016/B978-0-444-54299-1.00009-1.

The in vivo Down syndrome genomic library in mouse.

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Institut de Génétique et de Biologie Moléculaire et Cellulaire, Translational medicine and Neurogenetics program, IGBMC, CNRS, INSERM, Université de Strasbourg, UMR7104, UMR964, Illkirch, Strasbourg, France.


Mouse models are key elements to better understand the genotype-phenotype relationship and the physiopathology of Down syndrome (DS). Even though the mouse will never recapitulate the whole spectrum of intellectual disabilities observed in the DS, mouse models have been developed over the recent decades and have been used extensively to identify homologous genes or entire regions homologous to the human chromosome 21 (Hsa21) that are necessary or sufficient to induce DS cognitive features. In this chapter, we review the principal mouse DS models which have been selected and engineered over the years either for large genomic regions or for a few or a single gene of interest. Their analyses highlight the complexity of the genetic interactions that are involved in DS cognitive phenotypes and also strengthen the hypothesis on the multigenic nature of DS. This review also addresses future research challenges relative to the making of new models and their combination to go further in the characterization of candidates and modifier of the DS features.

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