Fiche publication
Date publication
janvier 2020
Journal
Progress in brain research
Auteurs
Membres identifiés du Cancéropôle Est :
Dr HERAULT Yann
Tous les auteurs :
Muñiz Moreno MDM, Brault V, Birling MC, Pavlovic G, Herault Y
Lien Pubmed
Résumé
The genotype-phenotype relationship and the physiopathology of Down Syndrome (DS) have been explored in the last 20 years with more and more relevant mouse models. From the early age of transgenesis to the new CRISPR/CAS9-derived chromosomal engineering and the transchromosomic technologies, mouse models have been key to identify homologous genes or entire regions homologous to the human chromosome 21 that are necessary or sufficient to induce DS features, to investigate the complexity of the genetic interactions that are involved in DS and to explore therapeutic strategies. In this review we report the new developments made, how genomic data and new genetic tools have deeply changed our way of making models, extended our panel of animal models, and increased our understanding of the neurobiology of the disease. But even if we have made an incredible progress which promises to make DS a curable condition, we are facing new research challenges to nurture our knowledge of DS pathophysiology as a neurodevelopmental disorder with many comorbidities during ageing.
Mots clés
Animal models, Behavior and cognition, Chromosomal engineering, Genome, Neurobiology of disease, Transchromosomic, Transgenesis
Référence
Prog. Brain Res.. 2020 ;251:91-143
