Hyper-IgM syndromes: a model for studying the regulation of class switch recombination and somatic hypermutation generation |
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Authors: | Durandy A |
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Affiliation: | Inserm U429, H?pital Necker-Enfants Malades, 149 Rue de Sèvres, Paris, France. durandy@necker.fr |
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Abstract: | Several genetic defects in class switch recombination, which lead to a hyper-IgM syndrome, have been described recently in humans. In addition to the well known role of CD40-ligand-CD40 interaction, these pathologies demonstrate definitively the requirement of CD40-mediated nuclear factor kappa B activation and the essential role of a recently described molecule, the activation-induced cytidine deaminase in an efficient humoral response, which includes class switch recombination and the production of high-affinity antibodies. |
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