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

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

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.

Publication types

  • Review

MeSH terms

  • Animals
  • CD40 Antigens / metabolism*
  • CD40 Ligand
  • Humans
  • Hypergammaglobulinemia / genetics*
  • Hypergammaglobulinemia / immunology*
  • Immunoglobulin M / blood*
  • Infant, Newborn
  • Membrane Glycoproteins / biosynthesis
  • Membrane Glycoproteins / metabolism*
  • Mice
  • Mice, Knockout
  • X Chromosome / genetics

Substances

  • CD40 Antigens
  • Immunoglobulin M
  • Membrane Glycoproteins
  • CD40 Ligand