Research ArticleIMMUNODEFICIENCIES

A recessive form of hyper-IgE syndrome by disruption of ZNF341-dependent STAT3 transcription and activity

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Science Immunology  15 Jun 2018:
Vol. 3, Issue 24, eaat4956
DOI: 10.1126/sciimmunol.aat4956

Article Information

vol. 3 no. 24

PubMed: 
Published By: 
History: 
  • Received March 5, 2018
  • Accepted May 17, 2018
  • .

Author Information

  1. Vivien Béziat1,2,*,
  2. Juan Li3,,
  3. Jian-Xin Lin4,,
  4. Cindy S. Ma5,6,,
  5. Peng Li4,,
  6. Aziz Bousfiha7,,
  7. Isabelle Pellier8,,
  8. Samaneh Zoghi9,10,11,,
  9. Safa Baris12,,
  10. Sevgi Keles13,,
  11. Paul Gray14,15,,
  12. Ning Du4,,
  13. Yi Wang1,2,,
  14. Yoann Zerbib1,2,,
  15. Romain Lévy1,2,,
  16. Thibaut Leclercq1,2,,
  17. Frédégonde About1,2,
  18. Ai Ing Lim16,17,
  19. Geetha Rao5,
  20. Kathryn Payne5,
  21. Simon J. Pelham5,6,
  22. Danielle T. Avery5,
  23. Elissa K. Deenick5,6,
  24. Bethany Pillay5,6,
  25. Janet Chou18,19,
  26. Romain Guery1,2,20,
  27. Aziz Belkadi1,2,
  28. Antoine Guérin1,2,
  29. Mélanie Migaud1,2,
  30. Vimel Rattina1,2,
  31. Fatima Ailal7,
  32. Ibtihal Benhsaien7,
  33. Matthieu Bouaziz1,2,
  34. Tanwir Habib21,
  35. Damien Chaussabel21,
  36. Nico Marr21,
  37. Jamel El-Benna22,
  38. Bodo Grimbacher23,
  39. Orli Wargon24,
  40. Jacinta Bustamante1,2,3,25,
  41. Bertrand Boisson1,2,3,
  42. Ingrid Müller-Fleckenstein26,
  43. Bernhard Fleckenstein26,
  44. Marie-Olivia Chandesris27,28,
  45. Matthias Titeux2,29,
  46. Sylvie Fraitag30,
  47. Marie-Alexandra Alyanakian31,
  48. Marianne Leruez-Ville32,33,
  49. Capucine Picard2,25,33,34,
  50. Isabelle Meyts35,
  51. James P. Di Santo16,17,
  52. Alain Hovnanian2,29,36,§,
  53. Ayper Somer37,§,
  54. Ahmet Ozen12,§,
  55. Nima Rezaei9,10,11,§,
  56. Talal A. Chatila18,19,,
  57. Laurent Abel1,2,3,,
  58. Warren J. Leonard4,,
  59. Stuart G. Tangye5,6,,
  60. Anne Puel1,2,3,*,** and
  61. Jean-Laurent Casanova1,2,3,34,38,*,**
  1. 1Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France.
  2. 2Paris Descartes University, Imagine Institute, 75015 Paris, France.
  3. 3St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY 10065, USA.
  4. 4Laboratory of Molecular Immunology and the Immunology Center, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892–1674, USA.
  5. 5Immunology Division, Garvan Institute of Medical Research, Darlinghurst, Sydney, New South Wales 2010, Australia.
  6. 6St. Vincent’s Clinical School, University of New South Wales, Sydney, New South Wales 2052, Australia.
  7. 7Clinical Immunology Unit, Casablanca Children’s Hospital, Ibn Rochd Medical School, King Hassan II University, Casablanca, Morocco.
  8. 8Pediatric Hemato-Oncology Unit, University Hospital of Angers, 49933 Angers, France.
  9. 9Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran 1417613151, Iran.
  10. 10Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran 1419733151, Iran.
  11. 11Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
  12. 12Marmara University School of Medicine, Department of Pediatrics, Division of Allergy and Immunology, 34899 Istanbul, Turkey.
  13. 13Necmettin Erbakan University, Meram Medical Faculty, Division of Pediatric Allergy and Immunology, 42060 Konya, Turkey.
  14. 14Department of Immunology and Infectious Diseases, Sydney Children’s Hospital, Randwick, New South Wales 2031, Australia.
  15. 15School of Women’s and Children’s Health, University of New South Wales School of Women’s and Children’s Health, Sydney, New South Wales 2031, Australia.
  16. 16Innate Immunity Unit, Institut Pasteur, 75015 Paris, France.
  17. 17INSERM U1223, 75015 Paris, France.
  18. 18Division of Immunology, Boston Children’s Hospital, Boston, MA 02115, USA.
  19. 19Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA.
  20. 20Unit of Tropical and Infectious Diseases, Necker Hospital for Sick Children, Assistance Publique–Hôpitaux de Paris (AP-HP), 75015 Paris, France.
  21. 21Sidra Medicine, Doha, Qatar.
  22. 22INSERM-U1149, CNRS-ERL8252, Center for Research on Inflammation, Labex Inflamex, Paris Diderot University, Faculté de Médecine, Xavier Bichat Medical School, 75018 Paris, France.
  23. 23Center for Chronic Immunodeficiency (CCI), Medical Center, Faculty of Medicine, University of Freiburg, 79106 Freiburg, Germany.
  24. 24Department of Paediatric Dermatology, Sydney Children’s Hospital, High Street, Randwick, New South Wales 2031, Australia.
  25. 25Study Center for Immunodeficiency, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.
  26. 26Institute of Clinical and Molecular Virology, University of Erlangen-Nürnberg, D-91054 Erlangen, Germany.
  27. 27Department of Hematology, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.
  28. 28Referral Center for Immunodeficiency, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.
  29. 29Laboratory of Genetic Skin Diseases: from Disease Mechanism to Therapies, INSERM U1163, 75015 Paris, France.
  30. 30Department of Pathology, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.
  31. 31Immunology Laboratory, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.
  32. 32Virology Laboratory, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.
  33. 33Paris Descartes University, EA 73-28, 75015 Paris, France.
  34. 34Pediatric Hematology-Immunology Unit, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.
  35. 35Department of Immunology and Microbiology, Childhood Immunology, Department of Pediatrics, University Hospitals Leuven and KU Leuven, 3000 Leuven, Belgium.
  36. 36Department of Genetics, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.
  37. 37Istanbul University, Istanbul Medical Faculty, Division of Infectious Diseases and Immunology, 34452 Istanbul, Turkey.
  38. 38Howard Hughes Medical Institute, New York, NY 10065, USA.
  1. *Corresponding author. Email: anne.puel{at}inserm.fr (A.P.); jean-laurent.casanova{at}rockefeller.edu (J.L.C.); vivien.beziat{at}inserm.fr (V.B.)
  1. These authors contributed equally to this work.

  2. These authors contributed equally to this work.

  3. § These authors contributed equally to this work.

  4. These authors contributed equally to this work.

  5. These authors contributed equally to this work.

  6. ** These authors contributed equally to this work.

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