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Impaired thymic AIRE expression underlies autoantibodies against type I IFNs in humans with inborn errors of the alternative NF-kB pathway
  • +112
  • Tom Le Voyer,
  • Adrian Gervais,
  • Jérémie Rosain,
  • Audrey Parent,
  • Axel Cederholm,
  • Darawan Rinchai,
  • Lucy Bizien,
  • Gonca Hancioglu,
  • Quentin Philippot,
  • Mame Sokhna Gueye,
  • Majistor Raj Luxman,
  • Maglorius Renkilaraj,
  • Masato Ogishi,
  • Camille Soudée,
  • Mélanie Migaud,
  • Flore Rozenberg,
  • Mana Momenilandi,
  • Quentin Riller,
  • Luisa Imberti,
  • Ottavia Delmonte,
  • Gabriele Müller,
  • Bärbel Keller,
  • Julio Orrego,
  • William Alexander Gallego,
  • Tamar Rubin,
  • Melike Emiroglu,
  • Nima Parvaneh,
  • Daniel Eriksson,
  • Maribel Aranda-Guillen,
  • David I Berrios,
  • Linda Vong,
  • Connie H Katelaris,
  • Peter Mustillo,
  • Johannes Rädler,
  • Jonathan Bohlen,
  • Jale Bengi Celik,
  • Camila Astudillo,
  • Sarah Winter,
  • Audrey Guichard,
  • Vivien Béziat,
  • Jacinta Bustamante,
  • Qiang Pan-Hammarström,
  • Yu Zhang,
  • Lindsey B Rosen,
  • Steve M Holland,
  • Heather Kenney,
  • Kaan Boztuğ,
  • Nizar Mahlaoui,
  • Sylvain Latour,
  • Roshini Abraham,
  • Vassilios Lougaris,
  • Fabian Hauck,
  • Anna Sediva,
  • Faranaz Atschekzei,
  • M Cecilia Poli,
  • Mary A Slatter,
  • Boaz Palterer,
  • Michael D Keller,
  • Alberto Pinzon-Charry,
  • Anna Sullivan,
  • Luke Droney,
  • Daniel Suan,
  • Nathalie Aladjidi,
  • Miguel Hie,
  • Estibaliz Lazaro,
  • Jose Franco,
  • Sevgi Keles,
  • Marion Malphette,
  • Marlene Pasquet,
  • Maria Elena Maccari,
  • Andrea Meinhardt,
  • Aydan Ikinciogullari,
  • Mohammad Shahrooei,
  • Fatih Celmeli,
  • Patrick Frosk,
  • Christopher C Goodnow,
  • Paul E Gray,
  • Alexandre Belot,
  • Hye Sun Kuehn,
  • Sergio D Rosenzweig,
  • Francesco Licciardi,
  • Amélie Servettaz,
  • Vincent Barlogis,
  • Guillaume Le Guenno,
  • Vera-Maria Herrmann,
  • Taco Kuijpers,
  • Grégoire Ducoux,
  • Françoise Sarrot-Reynauld,
  • Catharina Schuetz,
  • Charlotte Cunningham-Rundles,
  • Frédéric Rieux-Laucat,
  • Stuart G Tangye,
  • Cristina Sobacchi,
  • Rainer Doffinger,
  • Klaus Warnatz,
  • Bodo Grimbacher,
  • Claire Fieschi,
  • Laureline Berteloot,
  • Vanessa Bryant,
  • Sophie Trouillet Assant,
  • Luigi D Notarangelo,
  • Helen Su,
  • Benedicte Neven,
  • Laurent Abel,
  • Qian Zhang,
  • Bertrand Boisson,
  • Aurélie Cobat,
  • Emmanuelle Jouanguy,
  • Olle Kampe,
  • Paul Bastard,
  • Chaim Roifman,
  • Nils Landegren,
  • Mark S Anderson,
  • Jean-Laurent Casanova,
  • Anne Puel
Tom Le Voyer
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute

Corresponding Author:[email protected]

Author Profile
Adrian Gervais
University of Paris Cité, Imagine Institute, Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France
Jérémie Rosain
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Audrey Parent
Diabetes Center, University of California
Axel Cederholm
Cochin-Saint-Vincent de Paul Hospital, Division of Pediatric Allergy and Immunology, Ondokuz Mayıs University Faculty of Medicine, University of Paris, Science for Life Laboratory, Uppsala University
Darawan Rinchai
St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, United States
Lucy Bizien
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Gonca Hancioglu
Division of Pediatric Allergy and Immunology, Ondokuz Mayıs University Faculty of Medicine, Samsun, Turkey
Quentin Philippot
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Mame Sokhna Gueye
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Majistor Raj Luxman
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Maglorius Renkilaraj
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Masato Ogishi
St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, United States
Camille Soudée
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Mélanie Migaud
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Flore Rozenberg
Virology, Cochin-Saint-Vincent de Paul Hospital, University of Paris, Paris, France
Mana Momenilandi
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, Specialized Immunology Laboratory of Dr. Shahrooei, University of Paris Cité, Imagine Institute
Quentin Riller
Institut Imagine, INSERM UMR1163, Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Université de Paris
Luisa Imberti
Diagnostic Department, ASST Spedali Civili di Brescia, CREA Laboratory
Ottavia Delmonte
Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United Stated
Gabriele Müller
Institute for Immunodeficiency, Center for Chronic Immunodeficiencies, Medical Center -University Hospital Freiburg, Faculty of Medicine, Albert-Ludwigs-University
Bärbel Keller
Institute for Immunodeficiency, Center for Chronic Immunodeficiencies, Medical Center -University Hospital Freiburg, Faculty of Medicine, Albert-Ludwigs-University
Julio Orrego
Primary Immunodeficiencies Group, Department of Microbiology and Parasitology, School of Medicine, University of Antioquia, Medellín, Colombia
William Alexander Gallego
Primary Immunodeficiencies Group, Department of Microbiology and Parasitology, School of Medicine, University of Antioquia, Medellín, Colombia
Tamar Rubin
Division of Pediatric Clinical Immunology and Allergy, Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Canada
Melike Emiroglu
Department of Pediatric Infectious Diseases, Faculty of Medicine, Selcuk University, Konya, Turkey
Nima Parvaneh
Division of Allergy and Clinical Immunology, Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran
Daniel Eriksson
Center for Molecular Medicine, Department of Medicine (Solna), Karolinska Institutet, Stockholm 171 76, Sweden, Department of Immunology, Genetics and Pathology, Uppsala University and University Hospital, Section of Clinical Genetics, Uppsala, Sweden, Department of Clinical Genetics, Uppsala University Hospital, Uppsala, Sweden
Maribel Aranda-Guillen
Centre for Molecular Medicine, Department of Medicine (Solna), Karolinska Institutet, Stockholm, Sweden
David I Berrios
Diabetes Center, University of California
Linda Vong
The Canadian Centre for Primary Immunodeficiency and The Jeffrey Modell Research Laboratory for the Diagnosis of Primary Immunodeficiency, The Hospital for Sick Children, Toronto, ON, Canada, Division of Immunology and Allergy, Department of Paediatrics, Hospital for Sick Children and University of Toronto, Toronto, ON, Canada
Connie H Katelaris
Immunology and Allergy, University of Western Sydney and Campbelltown Hospital, NSW Australia
Peter Mustillo
Division of Allergy and Immuology, Nationwide Children’s Hospital, The Ohio State University College of Medicine, Comumbus, OH, United States
Johannes Rädler
Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität München, Munich, Germany
Jonathan Bohlen
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Jale Bengi Celik
Department of Anesthesiology and Reanimation, Selcuk University Faculty of Medicine, Konya, Turkey
Camila Astudillo
Department of Pediatrics, Facultad de Medicina Clinica Alemana Universidad del Desarrollo, Santiago, Chile
Sarah Winter
Laboratory of Lymphocyte Activation and Susceptibility to EBV, Imagine - Institut des Maladies Génétiques-Inserm UMR 1163, 24, boulevard du Montparnasse, 75015 Paris
Audrey Guichard
Joint unit Hospices Civils de Lyon – BioMérieux, Lyon, France
Vivien Béziat
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Jacinta Bustamante
Study Center of Immunodeficiencies, Necker Hospital for Sick Children, Paris, France, Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Qiang Pan-Hammarström
Department of Biosciences and Nutrition, Karolinska Institutet, 17177 Stockholm, Sweden
Yu Zhang
NIAID Clinical Genomics Program, NIH, Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, NIAID, NIH, Bethesda, MD, United States, Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United Stated
Lindsey B Rosen
Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United Stated
Steve M Holland
Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United Stated
Heather Kenney
NIAID Clinical Genomics Program, NIH, Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, NIAID, NIH, Bethesda, MD, United States
Kaan Boztuğ
CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria; Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
Nizar Mahlaoui
Department of Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades Hospital, APHP, Paris, France, French National Reference Center for Primary Immunodeficiencies (CEREDIH), Necker-Enfants University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
Sylvain Latour
Laboratory of Lymphocyte Activation and Susceptibility to EBV, Imagine - Institut des Maladies Génétiques-Inserm UMR 1163, 24, boulevard du Montparnasse, 75015 Paris
Roshini Abraham
Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Columbus, OH, United States
Vassilios Lougaris
Department of Clinical and Experimental Sciences, Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, University of Brescia ASST-Spedali Civili di Brescia, Brescia, Italy
Fabian Hauck
Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität München, Munich, Germany
Anna Sediva
Department of Immunology, Second Faculty of Medicine Charles University and Motol University Hospital, Prague, Czech Republic
Faranaz Atschekzei
Division of Rheumatology and Clinical Immunology, Hannover Medical University, Hannover, Germany
M Cecilia Poli
Hospital de Niños Dr. Roberto del Río, Santiago Chile
Mary A Slatter
Children's Haemopoietic Stem Cell Transplant Unit, Great North Children's Hospital, Newcastle upon Tyne Hospital NHS Foundation Trust, Newcastle upon Tyne, United Kingdom
Boaz Palterer
Allergy and Clinical Immunology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
Michael D Keller
Division of Allergy and Immunology, Children's National Medical Center, Washington, DC, United States
Alberto Pinzon-Charry
Clinical Immunogenomics Research Consortium Australasia (CIRCA), Darlinghurst, NSW Australia, Immunology and Allergy, Queensland Children's Hospital, Level 12, Queensland Children's Hospital, 501 Stanley Street, South Brisbane, QLD 4101, Australia
Anna Sullivan
Clinical Immunogenomics Research Consortium Australasia (CIRCA), Darlinghurst, NSW Australia, Immunology and Allergy, Queensland Children's Hospital, Level 12, Queensland Children's Hospital, 501 Stanley Street, South Brisbane, QLD 4101, Australia
Luke Droney
Immunology and Allergy, Queensland Children's Hospital, Level 12, Queensland Children's Hospital, 501 Stanley Street, South Brisbane, QLD 4101, Australia, Clinical Immunogenomics Research Consortium Australasia (CIRCA), Darlinghurst, NSW Australia
Daniel Suan
Westmead Clinical School, University of Sydney, Clinical Immunogenomics Research Consortium Australasia (CIRCA), Darlinghurst, NSW Australia
Nathalie Aladjidi
Pediatric Oncology Hematology Unit, University Hospital, Plurithématique CIC (CICP), Centre d'Investigation Clinique (CIC) 1401, Bordeaux, France
Miguel Hie
Internal Medicine Department, Pitié-Salpêtrière Hospital, Paris, France
Estibaliz Lazaro
Department of Internal Medicine & Infectious Diseases, Bordeaux Hospital University, Bordeaux, France
Jose Franco
Primary Immunodeficiencies Group, Department of Microbiology and Parasitology, School of Medicine, University of Antioquia, Medellín, Colombia
Sevgi Keles
Necmettin Erbakan University, Meram Medical Faculty, Division of Pediatric Allergy and Immunology, Konya, Turkey
Marion Malphette
Clinical Immunology Department, Saint Louis Hospital, Paris, France
Marlene Pasquet
Department of Pediatric Hematology, Toulouse University Hospital, Toulouse, France
Maria Elena Maccari
Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany, Institute for Immunodeficiency, Center for Chronic Immunodeficiencies, Medical Center -University Hospital Freiburg, Faculty of Medicine, Albert-Ludwigs-University
Andrea Meinhardt
Institute for Immunodeficiency, Center for Chronic Immunodeficiencies, Medical Center -University Hospital Freiburg, Faculty of Medicine, Albert-Ludwigs-University, Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany
Aydan Ikinciogullari
Department of Pediatric Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey
Mohammad Shahrooei
Specialized Immunology Laboratory of Dr. Shahrooei
Fatih Celmeli
Department of Allergy and Immunology, University of Medical Science, Antalya Education and Research Hospital, Antalya, Turkey
Patrick Frosk
Department of Biochemistry and Medical Genetics, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, MB, Canada
Christopher C Goodnow
School of Clinical Medicine, UNSW Medicine & Health, Darlinghurst 2010, NSW, Australia, Garvan Institute of Medical Research, Darlinghurst 2010, NSW, Sydney, Australia
Paul E Gray
Immunology and Infectious Diseases, Sydney Children's hospital Randwick, Western Sydney university, Campbelltown NSW, Australia
Alexandre Belot
CNRS UMR 5308, ENS, UCBL, Lyon, France; National Referee Centre for Rheumatic, and Autoimmune and Systemic Diseases in Children (RAISE), Lyon, France; Lyon; Immunopathology Federation LIFE, Hospices Civils de Lyon, Lyon, France
Hye Sun Kuehn
Immunology Service, Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, United States
Sergio D Rosenzweig
Immunology Service, Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, United States
Francesco Licciardi
Department of Pediatrics and Public Health, Università degli Studi di Torino, Turin, Italy
Amélie Servettaz
Internal Medicine, Clinical Immunology and Infectious Diseases Department, University Hospital Center, Reims, France
Vincent Barlogis
CHU Marseille, Hôpital La Timone, Service d'Hémato-oncologie Pédiatrique, Assistance Publique-Hôpitaux de Marseille, Marseille, France
Guillaume Le Guenno
Centre Hospitalier Universitaire Estaing, Clermont-Ferrand, France
Vera-Maria Herrmann
Institute of Human Genetics, University of Leipzig Medical Center, Leipzig, Germany
Taco Kuijpers
Department of Pediatric Immunology, Rheumatology and Infectious Diseases, Emma Children's Hospital, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Grégoire Ducoux
Department of Internal Medicine, Edouard Herriot Hospital, Lyon, France
Françoise Sarrot-Reynauld
Department of Internal Medicine, Hôpital Michallon, CHU de Grenoble Alpes, Grenoble, France
Catharina Schuetz
Department of Paediatrics, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
Charlotte Cunningham-Rundles
Departments of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, United States
Frédéric Rieux-Laucat
Institut Imagine, INSERM UMR1163, Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Université de Paris
Stuart G Tangye
School of Clinical Medicine, UNSW Medicine & Health, Darlinghurst 2010, NSW, Australia, Garvan Institute of Medical Research, Darlinghurst 2010, NSW, Sydney, Australia
Cristina Sobacchi
Humanitas Clinical and Research Institute, Rozzano, Italy; CNR-IRGB, Milan Unit, Milan, Italy
Rainer Doffinger
Department of Clinical Biochemistry and Immunology, Addenbrooke's Hospital, Cambridge, United Kingdom
Klaus Warnatz
Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany, Institute for Immunodeficiency, Center for Chronic Immunodeficiencies, Medical Center -University Hospital Freiburg, Faculty of Medicine, Albert-Ludwigs-University
Bodo Grimbacher
Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany, Institute for Immunodeficiency, Center for Chronic Immunodeficiencies, Medical Center -University Hospital Freiburg, Faculty of Medicine, Albert-Ludwigs-University
Claire Fieschi
Clinical Immunology Department, Saint Louis Hospital, Paris, France
Laureline Berteloot
Pediatric Radiology Department, Assistance Publique-Hôpitaux de Paris (AP-HP), Necker Hospital for Sick Children, Paris, France
Vanessa Bryant
Walter and Eliza Hall Institute, Melbourne, Parkville, Australia
Sophie Trouillet Assant
CIRI (Centre International de Recherche en Infectiologie), Université de Lyon, Université Claude Bernard Lyon 1, INSERM U1111, CNRS, UMR5308, ENS Lyon, Université Jean Monnet de Saint-Etienne, Lyon, France
Luigi D Notarangelo
NIAID Clinical Genomics Program, NIH, Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, NIAID, NIH, Bethesda, MD, United States, Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United Stated
Helen Su
NIAID Clinical Genomics Program, NIH, Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, NIAID, NIH, Bethesda, MD, United States, Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United Stated
Benedicte Neven
Department of Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades Hospital, APHP, Paris, France
Laurent Abel
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, United States, University of Paris Cité, Imagine Institute
Qian Zhang
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, United States, University of Paris Cité, Imagine Institute
Bertrand Boisson
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, United States, University of Paris Cité, Imagine Institute
Aurélie Cobat
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Emmanuelle Jouanguy
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute
Olle Kampe
Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm 171 76, Sweden
Paul Bastard
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, United States, University of Paris Cité, Imagine Institute
Chaim Roifman
The Canadian Centre for Primary Immunodeficiency and The Jeffrey Modell Research Laboratory for the Diagnosis of Primary Immunodeficiency, The Hospital for Sick Children, Toronto, ON, Canada, Division of Immunology and Allergy, Department of Paediatrics, Hospital for Sick Children and University of Toronto, Toronto, ON, Canada
Nils Landegren
Cochin-Saint-Vincent de Paul Hospital, Division of Pediatric Allergy and Immunology, Ondokuz Mayıs University Faculty of Medicine, University of Paris, Science for Life Laboratory, Uppsala University
Mark S Anderson
Diabetes Center, University of California
Jean-Laurent Casanova
St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, United States, Howard Hughes Medical Institute, New York, NY, United States, Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute

Corresponding Author:

Anne Puel
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Paris, France, University of Paris Cité, Imagine Institute

Corresponding Author:

Abstract

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.