A MOUSE MODEL FOR DOWN-SYNDROME EXHIBITS LEARNING AND BEHAVIOR DEFICITS

Trisomy 21 or Down syndrome (DS) is the most frequent genetic cause of mental retardation, affecting one in 800 live born human beings. Mice with segmental trisomy 16 (Ts65Dn mice) are at dosage imbalance for g enes corresponding to those on human chromosome 21q21-22.3-which inclu des the so-called DS 'critical region'. They do not show early-onset o f Alzheimer disease pathology; however, Ts65Dn mice do demonstrate imp aired performance in a complex learning task requiring the integration of visual and spatial information. The reproducibility of this phenot ype among Ts65Dn mice indicates that dosage imbalance for a gene or ge nes in this region contributes to this impairment. The corresponding d osage imbalance for the human homologues of these genes may contribute to cognitive deficits in DS.