title: Mouse models for Down syndrome-associated developmental cognitive disabilities.
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.
authors.affiliation: Children's Guild Foundation Down Syndrome Research Program and Department of Cancer Genetics, Roswell Park Cancer Institute, Buffalo, NY 14263, USA.
authors.forename: Chunhong
authors.initials: C
authors.lastname: Liu
authors.forename: Pavel V
authors.initials: PV
authors.lastname: Belichenko
authors.forename: Li
authors.initials: L
authors.lastname: Zhang
authors.forename: Dawei
authors.initials: D
authors.lastname: Fu
authors.forename: Alexander M
authors.initials: AM
authors.lastname: Kleschevnikov
authors.forename: Antonio
authors.initials: A
authors.lastname: Baldini
authors.forename: Stylianos E
authors.initials: SE
authors.lastname: Antonarakis
authors.forename: William C
authors.initials: WC
authors.lastname: Mobley
authors.forename: Y Eugene
authors.initials: YE
authors.lastname: Yu
mesh_terms: D000818:Animals; D003072:Cognition Disorders / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology; D002658:Developmental Disabilities / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology; D004195:Disease Models, Animal; D004314:Down Syndrome / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D051379:Mice