Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM
Article Abstract:
A tumor necrosis factor-related activation protein (TRAP) has been identified which may mediate the rare HIG1M immunodeficiency. The protein is the physiological ligand for CD40 and expressed exclusively on the surface of stimulated T cells. Crosslinking with CD40 on B cells is required for inducing B cells to switch the kind of immunoglobulin they produce. The gene for TRAP has been mapped to an X-chromosome location which has been identified as related to the X-chromosome linked immunodeficiency (HIGM1). Evidence is presented that mutations in the TRAP gene result in HIGM1.
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1993
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CD40 ligand mutations in X-linked immunodeficiency with hyper-IgM
Article Abstract:
Alterations to the gene responsible for expression of the CD40 ligand were observed in four male children with X-chromosome linked immunodeficiency with hyper-immunoglobulin (hyper-IgM). These alterations are the molecular basis for the defects observed in B-cell inability to switch production of immunoglobulin isotypes. The CD40 ligand is a membrane protein homologous to tumor necrosis factor and involved in the interaction of T and B cells responsible for switching B-cell immunoglobulin isotype production.
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1993
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ICOS is an inducible T-cell co-stimulator structurally and functionally related to CD28
Article Abstract:
T-cell-specific cell-surface receptors CD28 and CTLA-4 have important regulatory roles in the immune system. A third member of the family molecules, inducible co-stimulator (ICOS) has been identified. It is a homo-dimeric protein of relative molecular mass with the same potency as CD28. It enhances basic T-cell responses to foreign antigen, but has to be de novo induced on the T-cell surface
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1999
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