Abstract
We studied the ability of B lymphocytes from patients with X-linked hyper IgM syndrome (HIGM1) to be activated via the CD40 membrane receptor. HIGM1 is caused by a CD40 ligand gene mutation, leading to defective expression on the membrane of activated T lymphocytes. We found that triggering of B cells by an anti-CD40 monoclonal antibody or the soluble CD40 ligand plus interleukin (IL)-4 or IL-10 led to B cell proliferation and/or differentiation towards IgG, IgA and IgE secretion. This was reflected by transcription of C gamma, alpha and epsilon membrane isotype expression and IgG, IgA and IgE production. These results confirm the integrity of B cells in patients with the HIGM1 immunodeficiency and open up new therapeutic possibilities.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Antibodies, Monoclonal / immunology*
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Antigens, CD / immunology*
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Antigens, Differentiation, B-Lymphocyte / immunology*
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B-Lymphocytes / immunology*
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Base Sequence
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CD40 Antigens
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CD40 Ligand
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Child
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Child, Preschool
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Cytokines / pharmacology
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Genetic Linkage
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Humans
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Hypergammaglobulinemia / genetics
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Hypergammaglobulinemia / immunology*
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Immunoglobulin M / analysis
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Immunoglobulins / biosynthesis*
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Ligands
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Lymphocyte Activation
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Male
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Membrane Glycoproteins / immunology*
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Molecular Sequence Data
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X Chromosome
Substances
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Antibodies, Monoclonal
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Antigens, CD
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Antigens, Differentiation, B-Lymphocyte
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CD40 Antigens
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Cytokines
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Immunoglobulin M
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Immunoglobulins
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Ligands
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Membrane Glycoproteins
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CD40 Ligand