Mouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental Disabilities
Down syndrome (DS) is the most common genetic cause of mental disability. Based on the homology of Hsa21 and the murine chromosomes Mmu16, Mmu17 and Mmu10, several mouse models of DS have been developed. The most commonly used model, the Ts65Dn mouse, has been widely used to investigate the neural m...
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| Format: | Article |
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Wiley
2012-01-01
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| Series: | Neural Plasticity |
| Online Access: | http://dx.doi.org/10.1155/2012/584071 |
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| author | Noemí Rueda Jesús Flórez Carmen Martínez-Cué |
| author_facet | Noemí Rueda Jesús Flórez Carmen Martínez-Cué |
| author_sort | Noemí Rueda |
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| description | Down syndrome (DS) is the most common genetic cause of mental disability. Based on the homology of Hsa21 and the murine chromosomes Mmu16, Mmu17 and Mmu10, 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. |
| format | Article |
| id | doaj-art-8ae09d403fe2417bbb1e97857820b708 |
| institution | Kabale University |
| issn | 2090-5904 1687-5443 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Neural Plasticity |
| spelling | doaj-art-8ae09d403fe2417bbb1e97857820b7082025-08-20T03:37:34ZengWileyNeural Plasticity2090-59041687-54432012-01-01201210.1155/2012/584071584071Mouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental DisabilitiesNoemí Rueda0Jesús Flórez1Carmen Martínez-Cué2Department of Physiology and Pharmacology, Faculty of Medicine, University of Cantabria, Santander 39011, SpainDepartment of Physiology and Pharmacology, Faculty of Medicine, University of Cantabria, Santander 39011, SpainDepartment of Physiology and Pharmacology, Faculty of Medicine, University of Cantabria, Santander 39011, SpainDown syndrome (DS) is the most common genetic cause of mental disability. Based on the homology of Hsa21 and the murine chromosomes Mmu16, Mmu17 and Mmu10, 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.http://dx.doi.org/10.1155/2012/584071 |
| spellingShingle | Noemí Rueda Jesús Flórez Carmen Martínez-Cué Mouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental Disabilities Neural Plasticity |
| title | Mouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental Disabilities |
| title_full | Mouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental Disabilities |
| title_fullStr | Mouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental Disabilities |
| title_full_unstemmed | Mouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental Disabilities |
| title_short | Mouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental Disabilities |
| title_sort | mouse models of down syndrome as a tool to unravel the causes of mental disabilities |
| url | http://dx.doi.org/10.1155/2012/584071 |
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