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

• Published in: Blood Vol. 124; no. 24; pp. 3561 - 3571
• Main Authors: Maglione, Paul J., Simchoni, Noa, Black, Samuel, Radigan, Lin, Overbey, Jessica R., Bagiella, Emilia, Bussel, James B., Bossuyt, Xavier, Casanova, Jean-Laurent, Meyts, Isabelle, Cerutti, Andrea, Picard, Capucine, Cunningham-Rundles, Charlotte
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 04.12.2014; American Society of Hematology
• Subjects: Adolescent; Adult; Animals; Antibodies, Bacterial - genetics; Antibodies, Bacterial - immunology; Antigens, Bacterial - immunology; B-Lymphocytes - immunology; B-Lymphocytes - pathology; Bacterial Infections - genetics; Bacterial Infections - immunology; Bacterial Infections - pathology; Cells, Cultured; Child; Child, Preschool; Female; Humans; Immunobiology; Immunoglobulin G - immunology; Immunoglobulin M - genetics; Immunoglobulin M - immunology; Immunologic Deficiency Syndromes - genetics; Immunologic Deficiency Syndromes - immunology; Immunologic Deficiency Syndromes - pathology; Infant; Interleukin-1 Receptor-Associated Kinases - genetics; Interleukin-1 Receptor-Associated Kinases - immunology; Male; Middle Aged; Myeloid Differentiation Factor 88 - genetics; Myeloid Differentiation Factor 88 - immunology; Polysaccharides, Bacterial - immunology; Primary Immunodeficiency Diseases; Toll-Like Receptor 7 - genetics; Toll-Like Receptor 7 - immunology; Toll-Like Receptor 9 - genetics; Toll-Like Receptor 9 - immunology
• ISSN: 0006-4971; 1528-0020; 1528-0020
• DOI: 10.1182/blood-2014-07-587824

IRAK-4 and MyD88 deficiencies impair interleukin 1 receptor and Toll-like receptor (TLR) signaling and lead to heightened susceptibility to invasive bacterial infections. Individuals with these primary immunodeficiencies have fewer immunoglobulin M (IgM)+IgD+CD27+ B cells, a population that resemble...