Mouse Models of Cognitive Disorders in Trisomy 21: A Review
- 8 March 2006
- journal article
- review article
- Published by Springer Nature in Behavior Genetics
- Vol. 36 (3) , 387-404
- https://doi.org/10.1007/s10519-006-9056-9
Abstract
Trisomy 21 (TRS21) is the most frequent genetic cause of mental retardation. Although the presence of an extra copy of HSA21 is known to be at the origin of the syndrome, we do not know which 225 HSA21 genes have an effect on cognitive processes. Mouse models of TRS21 have been developed using syntenies between HSA21 and MMU16, MMU10 and MMU17. Available mouse models carry extra fragments of MMU16 or of HSA21 that cover all of HSA21 (chimeric HSA21) or MMU16 (Ts16); some carry large parts of MMU16 (Ts65Dn, Ts1Cje, Ms1Cje), while others have reduced contiguous fragments covering the D21S17-ETS2 region or single transfected genes. This offers a nest design strategy for deciphering cognitive (learning, memory and exploration) and associated brain abnormalities involving each of these chromosomal regions. This review confirms the crucial but not exclusive contribution of the D21S17-ETS2 region encompassing 16 genes to cognitive disorders.Keywords
This publication has 71 references indexed in Scilit:
- Functional Analysis of Genes Implicated in Down Syndrome: 1. Cognitive Abilities in Mice Transpolygenic for Down Syndrome Chromosomal Region-1 (DCR-1)Behavior Genetics, 2004
- Synaptic structural abnormalities in the Ts65Dn mouse model of down syndromeJournal of Comparative Neurology, 2004
- Chromosome 21 and Down syndrome: from genomics to pathophysiologyNature Reviews Genetics, 2004
- Transgenic Mouse In Vivo Library of Human Down Syndrome Critical Region 1Journal of Neuropathology and Experimental Neurology, 2004
- Harm Avoidance, Anxiety, and Response to Novelty in the Adolescent S-100β Transgenic Mouse: Role of Serotonin and Relevance to Down SyndromeNeuropsychopharmacology, 2003
- Spatial representation and attention in toddlers with Williams syndrome and Down syndromeNeuropsychologia, 2003
- Frontal cortex BDNF levels correlate with working memory in an animal model of Down syndromeBehavioural Brain Research, 2002
- Neurodevelopmental delay, motor abnormalities and cognitive deficits in transgenic mice overexpressing Dyrk1A (minibrain), a murine model of Down's syndromeHuman Molecular Genetics, 2001
- Discovery and genetic localization of Down syndrome cerebellar phenotypes using the Ts65Dn mouseHuman Molecular Genetics, 2000
- Behavioral phenotype of individuals with Down syndromeMental Retardation and Developmental Disabilities Research Reviews, 2000