Limbic Epileptogenesis in a Mouse Model of Fragile X Syndrome
文献类型：期刊论文
| 作者 | Xiong, Zhi-Qi
|
| 刊名 | CEREBRAL CORTEX
 |
| 出版日期 | 2009 |
| 卷号 | 19期号:7页码:1504-1514 |
| 关键词 | epilepsy FMRP kindling mGluR5 mossy fiber sprouting NMDA MENTAL-RETARDATION PROTEIN METABOTROPIC GLUTAMATE-RECEPTOR SELECTIVE RNA-BINDING LONG-TERM DEPRESSION KINDLING MODEL MESSENGER-RNAS ELECTRICAL STIMULATION KNOCKOUT MICE EEG FINDINGS EPILEPSY |
| ISSN号 | 1047-3211 |
| 通讯作者 | Xiong, ZQ (reprint author), Chinese Acad Sci, Inst Neurosci, Shanghai Inst Biol Sci, 320 Yue Yang Rd, Shanghai 200031, Peoples R China,xiongzhiqi@ion.ac.cn |
| 英文摘要 | Fragile X syndrome (FXS), caused by silencing of the Fmr1 gene, is the most common form of inherited mental retardation. Epilepsy is reported to occur in 20-25% of individuals with FXS. However, no overall increased excitability has been reported in Fmr1 knockout (KO) mice, except for increased sensitivity to auditory stimulation. Here, we report that kindling increased the expressions of Fmr1 mRNA and protein in the forebrain of wild-type (WT) mice. Kindling development was dramatically accelerated in Fmr1 KO mice, and Fmr1 KO mice also displayed prolonged electrographic seizures during kindling and more severe mossy fiber sprouting after kindling. The accelerated rate of kindling was partially repressed by inhibiting N-methyl-D-aspartic acid receptor (NMDAR) with MK-801 or mGluR5 receptor with 2-methyl-6-(phenylethynyl)-pyridine (MPEP). The rate of kindling development in WT was not effected by MPEP, however, suggesting that FMRP normally suppresses epileptogenic signaling downstream of metabolic glutamate receptors. Our findings reveal that FMRP plays a critical role in suppressing limbic epileptogenesis and predict that the enhanced susceptibility of patients with FXS to epilepsy is a direct consequence of the loss of an important homeostatic factor that mitigates vulnerability to excessive neuronal excitation. |
| 学科主题 | Neurosciences & Neurology |
| 收录类别 | SCI |
| 资助信息 | National Basic Research Program of China[2006CB806600]; Key State Research Program of China[2006CB943900]; National "863'' high-tech research and development program[2006AA02Z166]; The National Natural Science Foundation of China[30721004]; Chinese Academy of Sciences[KSCX2-YW-R-099]; Ministry of Science and Technology of the People's Republic of China[2006AA02Z166] |
| 语种 | 英语 |
| 公开日期 | 2012-07-13 |
| 源URL | [http://ir.sibs.ac.cn/handle/331001/1654]  |
| 专题 | 上海神经科学研究所_神经所(总) 上海神经科学研究所_疾病神经生物学研究组 |
| 推荐引用方式 GB/T 7714 | Xiong, Zhi-Qi. Limbic Epileptogenesis in a Mouse Model of Fragile X Syndrome[J]. CEREBRAL CORTEX,2009,19(7):1504-1514. |
| APA | Xiong, Zhi-Qi.(2009).Limbic Epileptogenesis in a Mouse Model of Fragile X Syndrome.CEREBRAL CORTEX,19(7),1504-1514. |
| MLA | Xiong, Zhi-Qi."Limbic Epileptogenesis in a Mouse Model of Fragile X Syndrome".CEREBRAL CORTEX 19.7(2009):1504-1514. |
入库方式： OAI收割
来源：上海神经科学研究所
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