Pathological Plasticity in Fragile X Syndrome
Deficits in neuronal plasticity are common hallmarks of many neurodevelopmental disorders. In the case of fragile-X syndrome (FXS), disruption in the function of a single gene, FMR1, results in a variety of neurological consequences directly related to problems with the development, maintenance, and...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Neural Plasticity |
| Online Access: | http://dx.doi.org/10.1155/2012/275630 |
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| author | Brandon S. Martin Molly M. Huntsman |
| author_facet | Brandon S. Martin Molly M. Huntsman |
| author_sort | Brandon S. Martin |
| collection | DOAJ |
| description | Deficits in neuronal plasticity are common hallmarks of many neurodevelopmental disorders. In the case of fragile-X syndrome (FXS), disruption in the function of a single gene, FMR1, results in a variety of neurological consequences directly related to problems with the development, maintenance, and capacity of plastic neuronal networks. In this paper, we discuss current research illustrating the mechanisms underlying plasticity deficits in FXS. These processes include synaptic, cell intrinsic, and homeostatic mechanisms both dependent on and independent of abnormal metabotropic glutamate receptor transmission. We place particular emphasis on how identified deficits may play a role in developmental critical periods to produce neuronal networks with permanently decreased capacity to dynamically respond to changes in activity central to learning, memory, and cognition in patients with FXS. Characterizing early developmental deficits in plasticity is fundamental to develop therapies that not only treat symptoms but also minimize the developmental pathology of the disease. |
| format | Article |
| id | doaj-art-2afe51137d5b43599c1368e54bb5d611 |
| 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-2afe51137d5b43599c1368e54bb5d6112025-02-03T05:47:10ZengWileyNeural Plasticity2090-59041687-54432012-01-01201210.1155/2012/275630275630Pathological Plasticity in Fragile X SyndromeBrandon S. Martin0Molly M. Huntsman1Center for Neuroscience Research, Children's National Medical Center, Washington, DC 20010, USACenter for Neuroscience Research, Children's National Medical Center, Washington, DC 20010, USADeficits in neuronal plasticity are common hallmarks of many neurodevelopmental disorders. In the case of fragile-X syndrome (FXS), disruption in the function of a single gene, FMR1, results in a variety of neurological consequences directly related to problems with the development, maintenance, and capacity of plastic neuronal networks. In this paper, we discuss current research illustrating the mechanisms underlying plasticity deficits in FXS. These processes include synaptic, cell intrinsic, and homeostatic mechanisms both dependent on and independent of abnormal metabotropic glutamate receptor transmission. We place particular emphasis on how identified deficits may play a role in developmental critical periods to produce neuronal networks with permanently decreased capacity to dynamically respond to changes in activity central to learning, memory, and cognition in patients with FXS. Characterizing early developmental deficits in plasticity is fundamental to develop therapies that not only treat symptoms but also minimize the developmental pathology of the disease.http://dx.doi.org/10.1155/2012/275630 |
| spellingShingle | Brandon S. Martin Molly M. Huntsman Pathological Plasticity in Fragile X Syndrome Neural Plasticity |
| title | Pathological Plasticity in Fragile X Syndrome |
| title_full | Pathological Plasticity in Fragile X Syndrome |
| title_fullStr | Pathological Plasticity in Fragile X Syndrome |
| title_full_unstemmed | Pathological Plasticity in Fragile X Syndrome |
| title_short | Pathological Plasticity in Fragile X Syndrome |
| title_sort | pathological plasticity in fragile x syndrome |
| url | http://dx.doi.org/10.1155/2012/275630 |
| work_keys_str_mv | AT brandonsmartin pathologicalplasticityinfragilexsyndrome AT mollymhuntsman pathologicalplasticityinfragilexsyndrome |