IRAK-4 and MyD88 deficiencies impair IgM responses against T-independent bacterial antigens

Author:

Maglione Paul J.1ORCID,Simchoni Noa1,Black Samuel1,Radigan Lin1,Overbey Jessica R.2,Bagiella Emilia2,Bussel James B.3,Bossuyt Xavier4,Casanova Jean-Laurent56,Meyts Isabelle78,Cerutti Andrea1,Picard Capucine596,Cunningham-Rundles Charlotte110

Affiliation:

1. Division of Clinical Immunology, Department of Medicine,

2. Department of Health Evidence and Policy,

3. Division of Hematology, Departments of Pediatrics and Medicine, Weill Medical College of Cornell University, New York, NY;

4. Experimental Laboratory Immunology, Department of Microbiology and Immunology, Catholic University of Leuven,

5. Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Santé et de la Recherche Médicale and

6. St Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY

7. KU Leuven – University of Leuven, Department of Microbiology and Immunology, Childhood Immunology, and

8. University Hospitals Leuven, Department of Pediatrics, Leuven, Belgium;

9. Study Center of Primary Immunodeficiencies and Pediatric Hematology-Immunology Unit, Necker Hospital, Assistance Publique – Hôpitaux de Paris, Paris, France; and

10. Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY;

Abstract

Key PointsHuman IRAK-4 and MyD88 deficiencies impair T-independent IgM production, including IgM recognizing bacterial antigens. T-independent IgM impairment by IRAK-4 and MyD88 deficiencies is linked to inadequacy of the IgM+IgD+CD27+ B-cell subset.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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