CD40- CD40配体（CD40L）相互作用与X连锁高IgM综合征（HIGMX-1）

CD40-CD40 ligand (CD40L) interactions and X-linked hyperIgM syndrome (HIGMX-1).

作者信息

Ramesh N, Morio T, Fuleihan R, Worm M, Horner A, Tsitsikov E, Castigli E, Geha R S

机构信息

Division of Immunology, Children's Hospital, Boston, Massachusetts 02115, USA.

出版信息

Clin Immunol Immunopathol. 1995 Sep;76(3 Pt 2):S208-13. doi: 10.1016/s0090-1229(95)90252-x.

DOI:10.1016/s0090-1229(95)90252-x

PMID:7554470

Abstract

Interactions between the B cell surface antigen CD40 and its ligand (CD40L) expressed on activated T cells play a critical role in isotype switching. This is illustrated by failure of isotype switching in patients with X-linked hyperIgM syndrome in whom the CD40L gene is mutated and by failure of isotype switching of CD40-deficient mice in response to T-cell-dependent antigens. We review these findings and discuss the signaling mechanisms of CD40 and the developmental control and transcriptional regulation of CD40L expression.

摘要

B细胞表面抗原CD40与其在活化T细胞上表达的配体（CD40L）之间的相互作用在免疫球蛋白同种型转换中起关键作用。这一点在X连锁高IgM综合征患者中得到了体现，这些患者的CD40L基因发生突变，导致同种型转换失败；同时也体现在CD40缺陷小鼠对T细胞依赖性抗原的同种型转换失败上。我们回顾了这些发现，并讨论了CD40的信号传导机制以及CD40L表达的发育控制和转录调控。