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J-GLOBAL ID：201702235332030801   整理番号：17A0470606

ヒト適応免疫先天性免疫の先天異常を救済する【Powered by NICT】

Human Adaptive Immunity Rescues an Inborn Error of Innate Immunity

著者 (56件)： Israel Laura (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Israel Laura (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Wang Ying (Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505, USA) ,  Bulek Katarzyna (Department of Immunology, Cleveland Clinic Foundation, Cleveland, OH 44106, USA) ,  Della Mina Erika (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Della Mina Erika (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Zhang Zhao (Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505, USA) ,  Pedergnana Vincent (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Pedergnana Vincent (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Chrabieh Maya (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Chrabieh Maya (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Lemmens Nicole A. (Medical Microbiology and Infectious Diseases, Erasmus Medical Center, 3015 Rotterdam, the Netherlands) ,  Sancho-Shimizu Vanessa (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Sancho-Shimizu Vanessa (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Sancho-Shimizu Vanessa (Department of Virology, Imperial College London, Norfolk Place, London W21 PG, UK) ,  Sancho-Shimizu Vanessa (Section for Paediatrics, Department of Medicine, Imperial College London, London W21 PG, UK) ,  Descatoire Marc (INSERM U1151-CNRS UMR 8253, Paris Descartes University, Necker-Enfants Malades Medical School-Broussais, 75014 Paris, France) ,  Lasseau Theo (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Lasseau Theo (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Israelsson Elisabeth (Benaroya Research Institute at Virginia Mason, Seattle, WA 98101, USA) ,  Lorenzo Lazaro (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Lorenzo Lazaro (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Yun Ling (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Yun Ling (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Belkadi Aziz (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Belkadi Aziz (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Moran Andrew (Greater Manchester Immunology Service, Manchester Royal Infirmary, Manchester, M13 9WL, UK) ,  Weisman Leonard E. (Baylor College of Medicine, Texas Children’s Hospital, Houston, TX 77030, USA) ,  Vandenesch Francois (French National Reference Center for Staphylococci, Hospices Civils of Lyon, 69677 Bron Cedex, France) ,  Batteux Frederic (Department of Biological Immunology, Cochin Hospital, 75679 Paris, France) ,  Weller Sandra (INSERM U1151-CNRS UMR 8253, Paris Descartes University, Necker-Enfants Malades Medical School-Broussais, 75014 Paris, France) ,  Levin Michael (Section for Paediatrics, Department of Medicine, Imperial College London, London W21 PG, UK) ,  Herberg Jethro (Section for Paediatrics, Department of Medicine, Imperial College London, London W21 PG, UK) ,  Abhyankar Avinash (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Prando Carolina (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Itan Yuval (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  van Wamel Willem J.B. (Medical Microbiology and Infectious Diseases, Erasmus Medical Center, 3015 Rotterdam, the Netherlands) ,  Picard Capucine (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Picard Capucine (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Picard Capucine (Pediatric Hematology-Immunology Unit, Assistance Publique Hopitaux de Paris, Necker Hospital, 75015 Paris, France) ,  Picard Capucine (Study Center for Primary Immunodeficiencies, AP-HP, Necker Hospital, 75015 Paris, France) ,  Abel Laurent (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Abel Laurent (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Abel Laurent (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Chaussabel Damien (Benaroya Research Institute at Virginia Mason, Seattle, WA 98101, USA) ,  Li Xiaoxia (Department of Immunology, Cleveland Clinic Foundation, Cleveland, OH 44106, USA) ,  Beutler Bruce (Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505, USA) ,  Arkwright Peter D. (University of Manchester, Royal Manchester Children’s Hospital, Manchester M13 9WL, UK) ,  Casanova Jean-Laurent (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Casanova Jean-Laurent (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Casanova Jean-Laurent (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA) ,  Casanova Jean-Laurent (Pediatric Hematology-Immunology Unit, Assistance Publique Hopitaux de Paris, Necker Hospital, 75015 Paris, France) ,  Casanova Jean-Laurent (Howard Hughes Medical Institute, New York, NY 10065, USA) ,  Puel Anne (Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France) ,  Puel Anne (Paris Descartes University, Imagine Institute, 75015 Paris, France) ,  Puel Anne (St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 10065, USA)
資料名： Cell (Cambridge, Mass.)  (Cell (Cambridge, Mass.))
巻： 168  号： 5  ページ： 789-800.e10  発行年： 2017年 
JST資料番号： A0707B  ISSN： 0092-8674  資料種別： 逐次刊行物 (A)
記事区分： 原著論文  発行国： アメリカ合衆国 (USA)  言語： 英語 (EN)

抄録/ポイント： 単一遺伝子障害の不完全な浸透度の分子的基礎は不明である。常染色体劣性TIRAP欠損を有する血縁者八名について述べた。生命を脅かすぶどう球菌疾患は発端者における小児期に発生し,他の七つのホモ接合体ではそうではなかった。すべてToll様受容体1/2(TLR1/2),TLR2/6およびTLR4アゴニストに対する反応は,全てのTIRAP欠損個体の線維芽細胞と白血球における障害されていた。しかし,TLR2/6作動薬に対する全血応答ブドウ球菌リポテイコ酸(LTA)は指標症例個々においてのみ消失した,LTA特異的抗体を欠いた唯一のファミリーメンバー(Abs)。欠損応答は,インターロイキン-1受容体関連キナーゼ4(IRAK 4)-欠乏個体にではなく,患者における逆抗LTAモノクローナル抗体(mAb)であった。抗LTA mAbもTIRAP欠損マウスではなく,TLR2あるいはMyD88におけるマクロファージ応答を救済した。,後天性抗LTA Absは遺伝性TIRAP欠損患者におけるブドウ球菌LTAへのTLR2依存性免疫を救ったことから,不完全な浸透度を説明した。複合TIRAPと抗LTA ab欠損は,この患者におけるぶどう球菌疾患の根底にある。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： *自然免疫 ,  マクロファージ ,  患者 ,  線維芽細胞 ,  インターロイキン1 ,  常染色体 ,  モノクローナル抗体 ,  Toll様受容体 ,  *ヒト
準シソーラス用語： MyD88 ,  アゴニスト ,  Toll様受容体4 ,  Toll様受容体2 ,  浸透度 ,  *適応免疫 , 【Automatic Indexing@JST】

著者キーワード (8件)： TIRAP ,  Toll様受容体 ,  黄色ブドウ球菌 ,  リポテイコ酸 ,  LTA ,  抗LTA抗体 ,  原発性免疫不全 ,  不完全臨床浸透度

分類 (2件)： 細胞生理一般  (EF02010S) ,  細胞膜の受容体  (EF04030C)

タイトルに関連する用語 (4件)： ヒト ,  適応免疫 ,  先天性免疫 ,  先天異常