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J-GLOBAL ID：201802217125576892   整理番号：18A0159151

患者特異的iPSCを用いたシャルコー・マリー・トゥース病1A型の病因モデリング【Powered by NICT】

Modeling the Pathogenesis of Charcot-Marie-Tooth Disease Type 1A Using Patient-Specific iPSCs

著者 (19件)： Shi Lei (Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, Guangdong Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China) ,  Shi Lei (Division of Neurosurgical Intensive Care Unit, Department of Critical Care Medicine, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China) ,  Huang Lihua (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  He Ruojie (Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, Guangdong Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China) ,  Huang Weijun (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  Wang Huiyan (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  Lai Xingqiang (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  Zou Zhengwei (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  Sun Jiaqi (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  Ke Qiong (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  Zheng Minying (Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, Guangdong Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China) ,  Lu Xilin (Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, Guangdong Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China) ,  Pei Zhong (Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, Guangdong Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China) ,  Su Huanxing (State Key Laboratory of Quality Research in Chinese Medicine, Institute of Chinese Medical Sciences, University of Macau, Macao 999078, China) ,  Xiang Andy Peng (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  Xiang Andy Peng (Department of Biochemistry, Zhongshan Medical School, Sun Yat-sen University, Guangzhou 510080, China) ,  Li Weiqiang (Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University, Guangzhou 510080, China) ,  Li Weiqiang (Department of Biochemistry, Zhongshan Medical School, Sun Yat-sen University, Guangzhou 510080, China) ,  Yao Xiaoli (Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, Guangdong Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China)
資料名： Stem Cell Reports  (Stem Cell Reports)
巻： 10  号： 1  ページ： 120-133  発行年： 2018年 
JST資料番号： W3132A  ISSN： 2213-6711  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： 最も頻度の高い遺伝性末梢神経障害の一つであるCharcot-Marie-Tooth病1A型(CMT1A)はPMP22遺伝子重複と関連している。CMT1Aのこれまでの研究は,主にげっ歯類モデルに依存し,PMP22過剰発現は患者の表現型をもたらすかはまだ明らかではない。ここでは,in vitro細胞モデルとして二CMT1A患者からのヒト人工多能性幹細胞(hiPSC)系統を作成した。,正常対照細胞と異なり,CMT1A hiPSCは神経冠幹細胞(NCSC)を介してSchwann細胞を生成したことは稀であることを見出した。代わりに,CMT1A NCSCsは,シュワン細胞分化系における多数の神経内膜線維芽細胞様細胞を生成し,同様の結果がPMP22を過剰発現するiPSCモデルで得られた。CMT1A病原性の脱髄再ミエリン化および/または髄鞘形成不全理論にもかかわらず,シュワン細胞の発達障害は,CMT1Aの根本的な原因と考えられる。著者らの結果は,基礎となる機構の解明とCMT1A神経障害に対する有望な治療戦略の開発に重要な意味を持つ可能性がある。Copyright 2018 Elsevier B.V., Amsterdam. All rights reserved. Translated from English into Japanese by JST.【Powered by NICT】

シソーラス用語： Schwann細胞 ,  齧歯類 ,  ニューロパチー ,  ヒト ,  病原性 ,  *モデリング ,  *患者 ,  iPS細胞 ,  表現型 ,  治療計画 ,  *病因
準シソーラス用語： 神経 ,  過剰発現 ,  PMP22 ,  PMP22遺伝子 , 【Automatic Indexing@JST】

著者キーワード (7件)： シャルコー・マリー・トゥース1A型 ,  人工多能性幹細胞 ,  神経冠幹細胞 ,  シュワン細胞 ,  神経内膜線維芽細胞様細胞 ,  differentiation ,  疾患モデリング

分類 (2件)： 神経の基礎医学  (GN01020W) ,  神経系の疾患  (GN03000O)

タイトルに関連する用語 (5件)： 患者 ,  IPSC ,  シャルコー・マリー・トゥース病 ,  病因 ,  モデリング