Hyper-IgM syndromes: a model for studying the regulation of class switch recombination and somatic hypermutation generation

被引:35
作者
Durandy, A [1 ]
机构
[1] Hop Necker Enfants Malad, INSERM, U429, Paris, France
关键词
activation-induced cytidine deaminase B-lymphocyte; maturation; CD40-CD40-ligand interaction; germinal centre;
D O I
10.1042/bst0300815
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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 kappaB 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.
引用
收藏
页码:815 / 818
页数:4
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