Hyper-IgM syndromes: a model for studying the regulation of class switch recombination and somatic hypermutation generation
2002 ◽
Vol 30
(4)
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pp. 815-818
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Keyword(s):
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 kB activation and the essential role of a recently described molecule, the activationinduced cytidine deaminase in an efficient humoral response, which includes class switch recombination and the production of high-affinity antibodies.