https://scholars.lib.ntu.edu.tw/handle/123456789/467127
標題: | RBFOX3/NeuN is Required for Hippocampal Circuit Balance and Function | 作者: | Wang H.-Y. Hsieh P.-F. Huang D.-F. Chin P.-S. Chou C.-H. Tung C.-C. Chen S.-Y. LI-JEN LEE SUSAN SHUR-FEN GAU HSIEN-SUNG HUANG |
公開日期: | 2015 | 出版社: | Nature Publishing Group | 卷: | 5 | 起(迄)頁: | 17383 | 來源出版物: | Scientific Reports | 摘要: | RBFOX3 mutations are linked to epilepsy and cognitive impairments, but the underlying pathophysiology of these disorders is poorly understood. Here we report replication of human symptoms in a mouse model with disrupted Rbfox3. Rbfox3 knockout mice displayed increased seizure susceptibility and decreased anxiety-related behaviors. Focusing on hippocampal phenotypes, we found Rbfox3 knockout mice showed increased expression of plasticity genes Egr4 and Arc, and the synaptic transmission and plasticity were defective in the mutant perforant pathway. The mutant dentate granules cells exhibited an increased frequency, but normal amplitude, of excitatory synaptic events, and this change was associated with an increase in the neurotransmitter release probability and dendritic spine density. Together, our results demonstrate anatomical and functional abnormality in Rbfox3 knockout mice, and may provide mechanistic insights for RBFOX3-related human brain disorders. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-84948953340&doi=10.1038%2fsrep17383&partnerID=40&md5=3bb84291f151536397f08e2d0eb62aee https://scholars.lib.ntu.edu.tw/handle/123456789/467127 |
ISSN: | 2045-2322 | DOI: | 10.1038/srep17383 | SDG/關鍵字: | activity regulated cytoskeletal-associated protein; cytoskeleton protein; early growth response factor; Egr4 protein, mouse; nerve protein; NeuN protein, mouse; nuclear protein; animal; anxiety; biosynthesis; cognitive defect; disease model; epilepsy; genetics; hippocampus; human; knockout mouse; metabolism; mouse; pathology; pathophysiology; synaptic transmission; Animals; Anxiety; Cognition Disorders; Cytoskeletal Proteins; Disease Models, Animal; Early Growth Response Transcription Factors; Epilepsy; Hippocampus; Humans; Mice; Mice, Knockout; Nerve Tissue Proteins; Nuclear Proteins; Synaptic Transmission |
顯示於: | 解剖學暨細胞生物學科所 |
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