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Mouse models for Down syndrome-associated developmental cognitive disabilities

Liu, Chunhong
Belichenko, Pavel V
Zhang, Li
Fu, Dawei
Kleschevnikov, Alexander M
Baldini, Antonio
Mobley, William C
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Published in Developmental Neuroscience. 2011, vol. 33, no. 5, p. 404-13
Abstract Down syndrome (DS) is mainly caused by the presence of an extra copy of human chromosome 21 (Hsa21) and is a leading genetic cause for developmental cognitive disabilities in humans. The mouse is a premier model organism for DS because the regions on Hsa21 are syntenically conserved with three regions in the mouse genome, which are located on mouse chromosome 10 (Mmu10), Mmu16 and Mmu17. With the advance of chromosomal manipulation technologies, new mouse mutants have been generated to mimic DS at both the genotypic and phenotypic levels. Further mouse-based molecular genetic studies in the future may lead to the unraveling of the mechanisms underlying DS-associated developmental cognitive disabilities, which would lay the groundwork for developing effective treatments for this phenotypic manifestation. In this review, we will discuss recent progress and future challenges in modeling DS-associated developmental cognitive disability in mice with an emphasis on hippocampus-related phenotypes.
Keywords AnimalsCognition Disorders/etiology/genetics/physiopathologyDevelopmental Disabilities/etiology/genetics/physiopathologyDisease Models, AnimalDown Syndrome/complications/genetics/physiopathologyHumansMice
PMID: 21865664
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Research group Pathologie Moléculaire de la Trisomie 21 et le Génome Humain (248)
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LIU, Chunhong et al. Mouse models for Down syndrome-associated developmental cognitive disabilities. In: Developmental Neuroscience, 2011, vol. 33, n° 5, p. 404-13. https://archive-ouverte.unige.ch/unige:34658

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Deposited on : 2014-03-07

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