Characterization of T and B cell repertoire diversity in patients with RAG deficiency

Author:

Lee Yu Nee12,Frugoni Francesco1,Dobbs Kerry3,Tirosh Irit1,Du Likun1,Ververs Francesca A.1,Ru Heng45,de Bruin Lisa Ott1,Adeli Mehdi6,Bleesing Jacob H.7,Buchbinder David8,Butte Manish J.9,Cancrini Caterina10,Chen Karin11,Choo Sharon12,Elfeky Reem A.13,Finocchi Andrea10,Fuleihan Ramsay L.14,Gennery Andrew R.1516,El-Ghoneimy Dalia H.13,Henderson Lauren A.1,Al-Herz Waleed17,Hossny Elham13,Nelson Robert P.18,Pai Sung-Yun19,Patel Niraj C.20,Reda Shereen M.13,Soler-Palacin Pere21,Somech Raz2,Palma Paolo10,Wu Hao45,Giliani Silvia2223,Walter Jolan E.124,Notarangelo Luigi D.3

Affiliation:

1. Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA 02115, USA.

2. Pediatric Department A and the Immunology Service, “Edmond and Lily Safra” Children’s Hospital, Jeffrey Modell Foundation Center, Sheba Medical Center, Tel Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

3. Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA.

4. Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, MA 02115, USA.

5. Program in Cellular and Molecular Medicine, Boston Children’s Hospital, Boston, MA 02115, USA.

6. Pediatrics Department, Weill Cornell Medical College, Hamad Medical Corporation, Doha, Qatar.

7. Division of Hematology/Oncology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USA.

8. Division of Hematology, Children’s Hospital Orange County, Orange County, CA 92868, USA.

9. Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, Stanford University, Stanford, CA 94305, USA.

10. DPUO, University Department of Pediatrics, Bambino Gesù Children’s Hospital and University of Tor Vergata School of Medicine, Rome, Italy.

11. Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT 84108, USA.

12. Department of Immunology, Royal Children’s Hospital, Melbourne, Victoria, Australia.

13. Department of Pediatric Allergy and Immunology, Children’s Hospital, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

14. Division of Allergy and Immunology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.

15. Department of Paediatric Immunology, Great North Children’s Hospital, Newcastle Upon Tyne, U.K.

16. Institute of Cellular Medicine, Newcastle University, Newcastle Upon Tyne, U.K.

17. Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait.

18. Division of Hematology and Oncology, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

19. Division of Hematology/Oncology, Boston Children’s Hospital, Boston, MA 02115, USA.

20. Division of Infectious Disease and Immunology, Department of Pediatrics, Levine Children’s Hospital, Carolinas Medical Center, Charlotte, NC 28203, USA.

21. Paediatric Infectious Diseases and Immunodeficiencies Unit, Vall d’Hebron University Hospital, Barcelona, Spain.

22. A. Nocivelli Institute for Molecular Medicine, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.

23. Section of Medical Genetics, Department of Pathology, Spedali Civili di Bresia, Brescia, Italy.

24. Division of Pediatric Allergy/Immunology, University of South Florida, and Johns Hopkins All Children’s Hospital, St. Petersburg, FL 33701, USA.

Abstract

Differences in B and T cell repertoires in patients with RAG deficiency associate with clinical severity.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

General Medicine,Immunology

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