Abstract
Aiolos encodes a zinc finger DNA-binding protein that is highly expressed in mature B cells and is homologous to Ikaros. In the periphery of mice homozygous for an Aiolos-null mutation, B cells exhibit an activated cell surface phenotype and undergo augmented antigen receptor (BCR)-mediated in vitro proliferative responses, even at limiting amounts of stimulant. In vivo, T cell-dependent B cell responses, including the formation of germinal centers and elevated serum IgG and IgE, are detected in Aiolos-deficient mice in the absence of immunization. Auto-antibodies and development of B cell lymphomas are frequently seen among aging Aiolos mutants. In sharp contrast to conventional B cells, B cells of the peritoneum, of the marginal zone, and the recirculating bone marrow population are greatly reduced.
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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Animals
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Autoantibodies / blood
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B-Lymphocytes / cytology
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B-Lymphocytes / immunology*
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Base Sequence
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Cell Differentiation
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Cytokines / biosynthesis
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DNA Primers / genetics
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DNA-Binding Proteins*
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Hematopoietic Stem Cells / cytology
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Hematopoietic Stem Cells / immunology
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Ikaros Transcription Factor
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Immunoglobulins / blood
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In Vitro Techniques
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Lymphocyte Activation
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Lymphoma, B-Cell / etiology
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Lymphoma, B-Cell / pathology
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Macromolecular Substances
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Mice
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Mice, Knockout
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Phenotype
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T-Lymphocytes / immunology
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Trans-Activators / chemistry
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Trans-Activators / genetics
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Trans-Activators / immunology*
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Transcription Factors / chemistry
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Transcription Factors / immunology
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Zinc Fingers / genetics
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Zinc Fingers / immunology
Substances
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Autoantibodies
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Cytokines
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DNA Primers
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DNA-Binding Proteins
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Ikzf3 protein, mouse
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Immunoglobulins
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Macromolecular Substances
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Trans-Activators
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Transcription Factors
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Zfpn1a1 protein, mouse
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Ikaros Transcription Factor