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J-GLOBAL ID：201702263090834694   整理番号：17A1627149

焦点性てんかんの基礎をなす脳回路の機能不全におけるCLOCKの消失【Powered by NICT】

Loss of CLOCK Results in Dysfunction of Brain Circuits Underlying Focal Epilepsy

著者 (35件)： Li Peijun (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Fu Xiaoqin (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Fu Xiaoqin (The Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325027, China) ,  Smith Nathan A. (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Ziobro Julie (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Curiel Julian (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Tenga Milagros J. (Virginia Tech Carillion Research Institute; Roanoke, VA 24014, USA) ,  Martin Brandon (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Freedman Samuel (Department of Pharmaceutical Sciences, Skaggs School of Pharmacy and Pharmaceutical Sciences, Department of Pediatrics, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA) ,  Cea-Del Rio Christian A. (Department of Pharmaceutical Sciences, Skaggs School of Pharmacy and Pharmaceutical Sciences, Department of Pediatrics, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA) ,  Oboti Livio (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Tsuchida Tammy N. (Comprehensive Pediatric Epilepsy Program, Division of Neurophysiology and Epilepsy, Children’s National Health Medical Center; Washington, DC 20010 USA) ,  Oluigbo Chima (Comprehensive Pediatric Epilepsy Program, Division of Neurophysiology and Epilepsy, Children’s National Health Medical Center; Washington, DC 20010 USA) ,  Oluigbo Chima (Division of Neurosurgery, Children’s National Medical Center, Washington, DC 20010, USA) ,  Yaun Amanda (Comprehensive Pediatric Epilepsy Program, Division of Neurophysiology and Epilepsy, Children’s National Health Medical Center; Washington, DC 20010 USA) ,  Yaun Amanda (Division of Neurosurgery, Children’s National Medical Center, Washington, DC 20010, USA) ,  Magge Suresh N. (Comprehensive Pediatric Epilepsy Program, Division of Neurophysiology and Epilepsy, Children’s National Health Medical Center; Washington, DC 20010 USA) ,  Magge Suresh N. (Division of Neurosurgery, Children’s National Medical Center, Washington, DC 20010, USA) ,  O’Neill Brent (Division of Pediatric Neurosurgery, School of Medicine, University of Colorado, Anschutz Medical Campus, Aurora, CO 80045, USA) ,  Kao Amy (Comprehensive Pediatric Epilepsy Program, Division of Neurophysiology and Epilepsy, Children’s National Health Medical Center; Washington, DC 20010 USA) ,  Zelleke Tesfaye G. (Comprehensive Pediatric Epilepsy Program, Division of Neurophysiology and Epilepsy, Children’s National Health Medical Center; Washington, DC 20010 USA) ,  Depositario-Cabacar Dewi T. (Comprehensive Pediatric Epilepsy Program, Division of Neurophysiology and Epilepsy, Children’s National Health Medical Center; Washington, DC 20010 USA) ,  Ghimbovschi Svetlana (Center for Genetic Medicine, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Knoblach Susan (Center for Genetic Medicine, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Ho Chen-Ying (Center for Genetic Medicine, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Ho Chen-Ying (Division of Pathology, Children’s National Medical Center; Washington, DC 20010, USA) ,  Corbin Joshua G. (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Goodkin Howard P. (Departments of Neurology and Pediatrics, University of Virginia Health System, Charlottesville, VA 22908, USA) ,  Vicini Stefano (Department of Pharmacology and Physiology, Georgetown University, Washington, DC 20057, USA) ,  Huntsman Molly M. (Department of Pharmaceutical Sciences, Skaggs School of Pharmacy and Pharmaceutical Sciences, Department of Pediatrics, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA) ,  Gaillard William D. (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA) ,  Gaillard William D. (Comprehensive Pediatric Epilepsy Program, Division of Neurophysiology and Epilepsy, Children’s National Health Medical Center; Washington, DC 20010 USA) ,  Valdez Gregorio (Virginia Tech Carillion Research Institute; Roanoke, VA 24014, USA) ,  Valdez Gregorio (Department of Biological Sciences, Virginia Tech; Blacksburg, VA 24061, USA) ,  Liu Judy S. (Center for Neuroscience Research, Children’s Research Institute, Children’s National Medical Center, Washington, DC 20010, USA)
資料名： Neuron  (Neuron)
巻： 96  号： 2  ページ： 387-401.e6  発行年： 2017年 
JST資料番号： T0649A  ISSN： 0896-6273  資料種別： 逐次刊行物 (A)
記事区分： 原著論文  発行国： オランダ (NLD)  言語： 英語 (EN)

抄録/ポイント： 難治性焦点てんかんの基礎となる分子機構はあまり明らかになっていないので,著者らはヒトてんかん原性組織のトランスクリプトーム解析を行った。対照と比べて,概日運動出力サイクルKaput(CLOCK)の発現はてんかん原性組織で減少した。CLOCKの機能を明らかにするため,Emx Creを作成し,試験した;クロック~flox/floxとPV-Cre;クロックは興奮性とパルブアルブミン(PV)発現抑制性ニューロンにおける時計遺伝子の標的欠失を持つflox/floxマウス系統を~であった。Emx Cre;クロック~flox/floxマウス系統単独ではけいれんいき値が,皮質には層流または樹状欠陥を減少させた。しかし,興奮性ニューロンEmx Cre;クロック~flox/floxマウスは自発的てんかん様放電を有していた。Emx Cre;からの両ニューロンクロック~flox/floxマウスおよびヒトてんかん原性組織は減少した自発的抑制性シナプス後電流を示した。最後に,Emx CreのビデオEEGクロック~flox/floxマウスは睡眠中のてんかん型放電と睡眠から生じる発作を明らかにした。まとめると,これらのデータはCLOCKの破壊は皮質回路を変化させ,焦点てんかんの発生につながる可能性があることを示した。Copyright 2017 Elsevier B.V., Amsterdam. All rights reserved. Translated from English into Japanese by JST.【Powered by NICT】

シソーラス用語： 興奮 ,  痙攣 ,  ヒト ,  *機能障害 ,  *てんかん ,  皮質 ,  パルブアルブミン ,  クロック ,  発作 ,  放電
準シソーラス用語： ビデオ脳波 ,  興奮性ニューロン ,  抑制性ニューロン ,  Cre ,  部分てんかん , 【Automatic Indexing@JST】

著者キーワード (4件)： 限局性皮質異形成 ,  結節性硬化症複合体 ,  概日てんかん ,  運動中枢損傷性てんかん

分類 (3件)： 中枢神経系  (EJ12030F) ,  神経の基礎医学  (GN01020W) ,  生物学的機能  (EB03040U)

タイトルに関連する用語 (5件)： 焦点性てんかん ,  基礎 ,  脳回 ,  機能不全 ,  CLOCK