Abstract
Autoreactive B cells are present in the lymphoid tissues of healthy individuals, but typically remain quiescent. When this homeostasis is perturbed, the formation of self-reactive antibodies can have serious pathological consequences. B cells expressing an antigen receptor specific for self-immunoglobulin-gamma (IgG) make a class of autoantibodies known as rheumatoid factor (RF). Here we show that effective activation of RF+ B cells is mediated by IgG2a-chromatin immune complexes and requires the synergistic engagement of the antigen receptor and a member of the MyD88-dependent Toll-like receptor (TLR) family. Inhibitor studies implicate TLR9. These data establish a critical link between the innate and adaptive immune systems in the development of systemic autoimmune disease and explain the preponderance of autoantibodies reactive with nucleic acid-protein particles. The unique features of this dual-engagement pathway should facilitate the development of therapies that specifically target autoreactive B cells.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Adaptor Proteins, Signal Transducing
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Animals
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Antigen-Antibody Complex
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Antigens, Differentiation / metabolism
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B-Lymphocytes / immunology*
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Chromatin / immunology*
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DNA-Binding Proteins / antagonists & inhibitors
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DNA-Binding Proteins / metabolism
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Deoxyribonuclease I / metabolism
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Drosophila Proteins*
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Immunoglobulin G / immunology*
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Immunoglobulin M / immunology*
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Lymphocyte Activation*
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Membrane Glycoproteins / metabolism*
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Mice
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Myeloid Differentiation Factor 88
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Receptors, Cell Surface / antagonists & inhibitors
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Receptors, Cell Surface / metabolism*
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Receptors, Complement 3b / deficiency
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Receptors, Complement 3b / immunology
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Receptors, Complement 3d / deficiency
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Receptors, Complement 3d / immunology
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Receptors, Immunologic / metabolism
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Rheumatoid Factor / immunology*
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Signal Transduction
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Toll-Like Receptor 9
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Toll-Like Receptors
Substances
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Adaptor Proteins, Signal Transducing
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Antigen-Antibody Complex
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Antigens, Differentiation
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Chromatin
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DNA-Binding Proteins
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Drosophila Proteins
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Immunoglobulin G
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Immunoglobulin M
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Membrane Glycoproteins
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Myd88 protein, mouse
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Myeloid Differentiation Factor 88
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Receptors, Cell Surface
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Receptors, Complement 3b
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Receptors, Complement 3d
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Receptors, Immunologic
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Tlr9 protein, mouse
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Toll-Like Receptor 9
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Toll-Like Receptors
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Rheumatoid Factor
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Deoxyribonuclease I