@article{10902/1020,
year = {2012},
month = {5},
url = {http://hdl.handle.net/10902/1020},
abstract = {Down syndrome (DS) is the most common genetic cause of mental disability. Based on the homology of Hsa21 and the murine chromosomesMmu16,Mmu17 andMmu10, several mouse models of DS have been developed. The most commonly used model, the Ts65Dn mouse, has been widely used to investigate the neural mechanisms underlying the mental disabilities seen in DS individuals. A wide array of neuromorphological alterations appears to compromise cognitive performance in trisomic mice. Enhanced inhibition due to alterations in GABAA-mediated transmission and disturbances in the glutamatergic, noradrenergic and cholinergic systems, among others, has also been demonstrated. DS cognitive dysfunction caused by neurodevelopmental alterations is worsened in later life stages by neurodegenerative processes. A number of pharmacological therapies have been shown to partially restore morphological anomalies concomitantly with cognition in these mice. In conclusion, the use of mouse models is enormously effective in the study of the neurobiological substrates of mental disabilities in DS and in the testing of therapies that rescue these alterations. These studies provide the basis for developing clinical trials in DS individuals and sustain the hope that some of these drugs will be useful in rescuing mental disabilities in DS individuals.},
organization = {This work was supported by the Jerome Lejeune Foundation and the Spanish Ministry of Innovation and Science
(BFU2008-04397 and BFU2011-24755).},
publisher = {Hindawi Publishing Corporation},
publisher = {Neural Plasticity, 2012, 2012, 584071},
title = {Mouse models of Down syndrome as a tool to unravel the causes of mental disabilities},
author = {Rueda Revilla, Noemí and Flórez Beledo, Jesús and Martínez-Cué, Carmen},
}