CD40 ligand and its role in X-linked hyper-IgM syndrome

Immunol Today. 1993 Nov;14(11):559-64. doi: 10.1016/0167-5699(93)90188-Q.

Abstract

CD40 ligand (CD40L) on activated T cells binding to CD40 on B cells is of critical importance for Ig heavy-chain switching and rescue of B cells from apoptosis after somatic mutation in the germinal centre. Mutations in the CD40L gene are now known to cause X-linked hyper-IgM syndrome (HIGM1), an immunodeficiency characterized by the absence of serum IgG, IgA and IgE. In this review, we discuss how basic and clinical immunology have combined to provide major insights into the function of CD40 in T-B cell collaboration.

Publication types

  • Review

MeSH terms

  • Antigens, CD / immunology*
  • Antigens, Differentiation, B-Lymphocyte / immunology*
  • B-Lymphocytes / immunology
  • CD40 Antigens
  • Genetic Linkage*
  • Genetic Therapy
  • Humans
  • Hypergammaglobulinemia / genetics
  • Hypergammaglobulinemia / immunology*
  • Immunoglobulin M / genetics
  • Immunoglobulin M / immunology*
  • Immunologic Deficiency Syndromes / immunology
  • Ligands
  • Lymphocyte Activation / immunology
  • Syndrome
  • T-Lymphocytes / immunology
  • X Chromosome*

Substances

  • Antigens, CD
  • Antigens, Differentiation, B-Lymphocyte
  • CD40 Antigens
  • Immunoglobulin M
  • Ligands