Mouse models of Down syndrome as a tool to unravel the causes of mental disabilities
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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.
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