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

Author:

Béziat Vivien12ORCID,Li Juan3,Lin Jian-Xin4,Ma Cindy S.56,Li Peng4,Bousfiha Aziz7,Pellier Isabelle8,Zoghi Samaneh91011,Baris Safa12,Keles Sevgi13,Gray Paul1415,Du Ning4,Wang Yi12ORCID,Zerbib Yoann12ORCID,Lévy Romain12ORCID,Leclercq Thibaut12,About Frédégonde12ORCID,Lim Ai Ing1617,Rao Geetha5,Payne Kathryn5ORCID,Pelham Simon J.56ORCID,Avery Danielle T.5,Deenick Elissa K.56ORCID,Pillay Bethany56,Chou Janet1819,Guery Romain1220,Belkadi Aziz12,Guérin Antoine12ORCID,Migaud Mélanie12ORCID,Rattina Vimel12ORCID,Ailal Fatima7,Benhsaien Ibtihal7,Bouaziz Matthieu12,Habib Tanwir21ORCID,Chaussabel Damien21ORCID,Marr Nico21ORCID,El-Benna Jamel22,Grimbacher Bodo23ORCID,Wargon Orli24,Bustamante Jacinta12325,Boisson Bertrand123,Müller-Fleckenstein Ingrid26,Fleckenstein Bernhard26,Chandesris Marie-Olivia2728,Titeux Matthias229ORCID,Fraitag Sylvie30,Alyanakian Marie-Alexandra31,Leruez-Ville Marianne3233ORCID,Picard Capucine2253334,Meyts Isabelle35,Di Santo James P.1617ORCID,Hovnanian Alain22936ORCID,Somer Ayper37ORCID,Ozen Ahmet12,Rezaei Nima91011,Chatila Talal A.1819ORCID,Abel Laurent123,Leonard Warren J.4ORCID,Tangye Stuart G.56ORCID,Puel Anne123ORCID,Casanova Jean-Laurent1233438ORCID

Affiliation:

1. Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, 75015 Paris, France.

2. Paris Descartes University, Imagine Institute, 75015 Paris, France.

3. St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY 10065, USA.

4. Laboratory of Molecular Immunology and the Immunology Center, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892–1674, USA.

5. Immunology Division, Garvan Institute of Medical Research, Darlinghurst, Sydney, New South Wales 2010, Australia.

6. St. Vincent’s Clinical School, University of New South Wales, Sydney, New South Wales 2052, Australia.

7. Clinical Immunology Unit, Casablanca Children’s Hospital, Ibn Rochd Medical School, King Hassan II University, Casablanca, Morocco.

8. Pediatric Hemato-Oncology Unit, University Hospital of Angers, 49933 Angers, France.

9. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran 1417613151, Iran.

10. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran 1419733151, Iran.

11. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.

12. Marmara University School of Medicine, Department of Pediatrics, Division of Allergy and Immunology, 34899 Istanbul, Turkey.

13. Necmettin Erbakan University, Meram Medical Faculty, Division of Pediatric Allergy and Immunology, 42060 Konya, Turkey.

14. Department of Immunology and Infectious Diseases, Sydney Children’s Hospital, Randwick, New South Wales 2031, Australia.

15. School 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. Innate Immunity Unit, Institut Pasteur, 75015 Paris, France.

17. INSERM U1223, 75015 Paris, France.

18. Division of Immunology, Boston Children’s Hospital, Boston, MA 02115, USA.

19. Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA.

20. Unit of Tropical and Infectious Diseases, Necker Hospital for Sick Children, Assistance Publique–Hôpitaux de Paris (AP-HP), 75015 Paris, France.

21. Sidra Medicine, Doha, Qatar.

22. INSERM-U1149, CNRS-ERL8252, Center for Research on Inflammation, Labex Inflamex, Paris Diderot University, Faculté de Médecine, Xavier Bichat Medical School, 75018 Paris, France.

23. Center for Chronic Immunodeficiency (CCI), Medical Center, Faculty of Medicine, University of Freiburg, 79106 Freiburg, Germany.

24. Department of Paediatric Dermatology, Sydney Children’s Hospital, High Street, Randwick, New South Wales 2031, Australia.

25. Study Center for Immunodeficiency, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.

26. Institute of Clinical and Molecular Virology, University of Erlangen-Nürnberg, D-91054 Erlangen, Germany.

27. Department of Hematology, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.

28. Referral Center for Immunodeficiency, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.

29. Laboratory of Genetic Skin Diseases: from Disease Mechanism to Therapies, INSERM U1163, 75015 Paris, France.

30. Department of Pathology, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.

31. Immunology Laboratory, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.

32. Virology Laboratory, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.

33. Paris Descartes University, EA 73-28, 75015 Paris, France.

34. Pediatric Hematology-Immunology Unit, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.

35. Department of Immunology and Microbiology, Childhood Immunology, Department of Pediatrics, University Hospitals Leuven and KU Leuven, 3000 Leuven, Belgium.

36. Department of Genetics, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France.

37. Istanbul University, Istanbul Medical Faculty, Division of Infectious Diseases and Immunology, 34452 Istanbul, Turkey.

38. Howard Hughes Medical Institute, New York, NY 10065, USA.

Abstract

ZNF341 is a newly characterized transcription factor controlling baseline and inducible transcription of the human STAT3 gene.

Funder

Foundation for the National Institutes of Health

Agence Nationale de la Recherche

Institut national de la santé et de la recherche médicale

Fondation pour la Recherche Médicale

Agence Nationale de Recherches sur le Sida et les Hepatites Virales

Publisher

American Association for the Advancement of Science (AAAS)

Subject

General Medicine,Immunology

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