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.
Keywords:
activation-induced cytidine deaminase B-lymphocyte maturation,
CD40-CD40-ligand interaction,
germinal centre,
AID, activation-induced cytidine deaminase,
apoB, apolipoprotein B,
APOBEC-I, apoB RNA-editing enzyme I,
C region, Ig constant region,
CD40-L, CD40-ligand,
CSR, class switch recombination,
ds, double-stranded
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© 2002 Biochemical Society
2002
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