CD40 ligand and its role in X-linked hyper-IgM syndrome.
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.
Callard
R E
RE
Cellular Immunology Unit, Institute of Child Health, London, UK.
Armitage
R J
RJ
Fanslow
W C
WC
Spriggs
M K
MK
D015703:Antigens, CD / Q000276:immunology*; D000944:Antigens, Differentiation, B-Lymphocyte / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology; D019013:CD40 Antigens; D008040:Genetic Linkage; D015316:Genetic Therapy; D006801:Humans; D006942:Hypergammaglobulinemia / Q000235:genetics / Q000276:immunology*; D007075:Immunoglobulin M / Q000235:genetics / Q000276:immunology*; D007153:Immunologic Deficiency Syndromes / Q000276:immunology; D008024:Ligands; D008213:Lymphocyte Activation / Q000276:immunology; D013577:Syndrome; D013601:T-Lymphocytes / Q000276:immunology; D014960:X Chromosome
D015703:Antigens, CD; D000944:Antigens, Differentiation, B-Lymphocyte; D019013:CD40 Antigens; D007075:Immunoglobulin M; D008024:Ligands