Impaired GABA Neural Circuits Are Critical for Fragile X Syndrome
Fragile X syndrome (FXS) is an inheritable neuropsychological disease caused by silence of the fmr1 gene and the deficiency of Fragile X mental retardation protein (FMRP). Patients present neuronal alterations that lead to severe intellectual disability and altered sleep rhythms. However, the neural...
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| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Neural Plasticity |
| Online Access: | http://dx.doi.org/10.1155/2018/8423420 |
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| author | Fei Gao Lijun Qi Zhongzhen Yang Tao Yang Yan Zhang Hui Xu Huan Zhao |
| author_facet | Fei Gao Lijun Qi Zhongzhen Yang Tao Yang Yan Zhang Hui Xu Huan Zhao |
| author_sort | Fei Gao |
| collection | DOAJ |
| description | Fragile X syndrome (FXS) is an inheritable neuropsychological disease caused by silence of the fmr1 gene and the deficiency of Fragile X mental retardation protein (FMRP). Patients present neuronal alterations that lead to severe intellectual disability and altered sleep rhythms. However, the neural circuit mechanisms underlying FXS remain unclear. Previous studies have suggested that metabolic glutamate and gamma-aminobutyric acid (GABA) receptors/circuits are two counter-balanced factors involved in FXS pathophysiology. More and more studies demonstrated that attenuated GABAergic circuits in the absence of FMRP are critical for abnormal progression of FXS. Here, we reviewed the changes of GABA neural circuits that were attributed to intellectual-deficient FXS, from several aspects including deregulated GABA metabolism, decreased expressions of GABA receptor subunits, and impaired GABAergic neural circuits. Furthermore, the activities of GABA neural circuits are modulated by circadian rhythm of FMRP metabolism and reviewed the abnormal condition of FXS mice or patients. |
| format | Article |
| id | doaj-art-aa869a18d3ca47e0b7fdcb76fd44dd60 |
| institution | Kabale University |
| issn | 2090-5904 1687-5443 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Neural Plasticity |
| spelling | doaj-art-aa869a18d3ca47e0b7fdcb76fd44dd602025-02-03T01:22:12ZengWileyNeural Plasticity2090-59041687-54432018-01-01201810.1155/2018/84234208423420Impaired GABA Neural Circuits Are Critical for Fragile X SyndromeFei Gao0Lijun Qi1Zhongzhen Yang2Tao Yang3Yan Zhang4Hui Xu5Huan Zhao6Department of Anesthesiology, Heze Municipal Hospital, Heze, 274031 Shandong, ChinaDepartment of Anesthesiology, Heze Municipal Hospital, Heze, 274031 Shandong, ChinaDepartment of Anesthesiology, Heze Municipal Hospital, Heze, 274031 Shandong, ChinaDepartment of Pain Treatment, Tangdu Hospital, Fourth Military Medical University, Xi'an 710038, ChinaDepartment of Anesthesiology, Heze Municipal Hospital, Heze, 274031 Shandong, ChinaDepartment of Neurobiology and Collaborative Innovation Center for Brain Science, School of Basic Medicine, Fourth Military Medical University, Xi'an 710032, ChinaDepartment of Anesthesiology, Heze Municipal Hospital, Heze, 274031 Shandong, ChinaFragile X syndrome (FXS) is an inheritable neuropsychological disease caused by silence of the fmr1 gene and the deficiency of Fragile X mental retardation protein (FMRP). Patients present neuronal alterations that lead to severe intellectual disability and altered sleep rhythms. However, the neural circuit mechanisms underlying FXS remain unclear. Previous studies have suggested that metabolic glutamate and gamma-aminobutyric acid (GABA) receptors/circuits are two counter-balanced factors involved in FXS pathophysiology. More and more studies demonstrated that attenuated GABAergic circuits in the absence of FMRP are critical for abnormal progression of FXS. Here, we reviewed the changes of GABA neural circuits that were attributed to intellectual-deficient FXS, from several aspects including deregulated GABA metabolism, decreased expressions of GABA receptor subunits, and impaired GABAergic neural circuits. Furthermore, the activities of GABA neural circuits are modulated by circadian rhythm of FMRP metabolism and reviewed the abnormal condition of FXS mice or patients.http://dx.doi.org/10.1155/2018/8423420 |
| spellingShingle | Fei Gao Lijun Qi Zhongzhen Yang Tao Yang Yan Zhang Hui Xu Huan Zhao Impaired GABA Neural Circuits Are Critical for Fragile X Syndrome Neural Plasticity |
| title | Impaired GABA Neural Circuits Are Critical for Fragile X Syndrome |
| title_full | Impaired GABA Neural Circuits Are Critical for Fragile X Syndrome |
| title_fullStr | Impaired GABA Neural Circuits Are Critical for Fragile X Syndrome |
| title_full_unstemmed | Impaired GABA Neural Circuits Are Critical for Fragile X Syndrome |
| title_short | Impaired GABA Neural Circuits Are Critical for Fragile X Syndrome |
| title_sort | impaired gaba neural circuits are critical for fragile x syndrome |
| url | http://dx.doi.org/10.1155/2018/8423420 |
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