Impaired thymic AIRE expression underlies autoantibodies against type I IFNs in humans with inborn errors of the alternative NF-kB pathway Tom Le Voyer , Adrian Gervais , Jérémie Rosain , Audrey Parent , Axel Cederholm , Darawan Rinchai , Lucy Bizien , Gonca Hancıoğlu , Quentin Philippot , Moctar Gueye , Majistor Raj Luxman , Maglorius Renkilaraj , Masato Ogishi , Camille Soudée , Mélanie Migaud , Flore Rozenberg , Mana Momenilandi , Quentin Riller , Luisa Imberti , Ottavia M. Delmonte , Gabriele Müller , Bärbel Keller , Julio César Orrego , William Alexander Franco Gallego , Tamar Rubin , Melike Emiroğlu , Nima Parvaneh , Daniel Eriksson , Maribel Aranda‐Guillén , David I. Berrios , Linda Vong , Connie Katelaris , Peter Mustillo , Johannes Rädler , Jonathan Bohlen , Jale Bengi Çelik , Camila Astudillo , Sarah Winter , Audrey Guichard , Vivien Béziat , Jacinta Bustamante , Qiang Pan‐Hammarström , Yu Zhang , Lindsey B. Rosen , Steve Holland , Heather Kenney , Kaan Boztuğ , Nizar Mahlaoui , Sylvain Latour , Roshini S. Abraham , Vassilios Lougaris , Fabian Hauck , Anna Šedivá , Faranaz Atschekzei , Cecilia Poli , Mary Slatter , Boaz Palterer , Michael D. Keller , Alberto Pinzón-Charry , Anna Sullivan , Luke Droney , Daniel Suan , Nathalie Aladjidi , M. Hié , Estibaliz Lazaro , José Roberto Fernández Franco , Sevgi Keleş , Marion Malphette , Marléne Pasquet , Maria Elena Maccari , Andrea Meinhardt , Aydan İkincioğulları , Mohammad Shahrooei , Fatih Çelmeli , Patrick Frosk , Christopher C. Goodnow , Paul Gray , Alexandre Belot , Hye Sun Kuehn , Sergio D. Rosenzweig , Francesco Licciardi , Amélie Servettaz , Vincent Barlogis , G. Le Guenno , Vera-Maria Herrmann , Taco Kuijpers , Grégoire Ducoux , F. Sarrot-Reynauld , Catharina Schuetz , Charlotte Cunningham‐Rundles , Frédéric Rieux‐Laucat , Stuart G. Tangye , Cristina Sobacchi , Rainer Döffinger , Klaus Warnatz , Bodo Grimbacher , Claire Fieschi , Laureline Berteloot , Vanessa L. Bryant , Sophie Trouillet‐Assant , Luigi D. Notarangelo , Helen C. Su , Bénédicte Neven , Laurent Abel , Qian Zhang , Bertrand Boisson , Aurélie Cobat , Emmanuelle Jouanguy , Olle Kämpe , Paul Bastard , Chaim M. Roifman , Nils Landegren , Mark S. Anderson , Jean‐Laurent Casanova , Anne Puel Authorea (Authorea)（2023）
摘要
Patients with inborn errors of the alternative NF-κB pathway have low thymic AIRE expression, leading to the development of auto-Abs neutralizing type I IFNs, and severe viral diseases.
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关键词
impaired thymic impaired expression, autoantibodies, inborn errors
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