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Title: Limbic Epileptogenesis in a Mouse Model of Fragile X Syndrome
Author: Qiu, Li-Feng ; Lu, Ting-Jia ; Hu, Xiao-Ling ; Yi, Yong-Hong ; Liao, Wei-Ping ; null(熊志奇)
Source: CEREBRAL CORTEX
Issued Date: 2009
Volume: 19, Issue:7, Pages:1504-1514
Keyword: 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
Subject: Neurosciences & Neurology
Corresponding Author: 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
English Abstract: 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.
Indexed Type: sci
Language: 英语
Content Type: 期刊论文
URI: http://ir.sibs.ac.cn/handle/331001/1654
Appears in Collections:神经所(总)_期刊论文
疾病神经生物学研究组_期刊论文

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Qiu, Li-Feng; Lu, Ting-Jia; Hu, Xiao-Ling; Yi, Yong-Hong; Liao, Wei-Ping; Xiong, Zhi-Qi.Limbic Epileptogenesis in a Mouse Model of Fragile X Syndrome,CEREBRAL CORTEX,2009,19(7):1504-1514
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