Article
Defects of T-cell effector function and post-thymic maturation in X-linked hyper-IgM syndrome.
The Journal of clinical investigation - 1 Apr 1999
Jain A, Atkinson T P, Lipsky P E, Slater J E, Nelson D L, Strober W
Abstract excerpt
X-linked hyper-IgM syndrome (XHIM) results from mutations in the gene encoding for CD40 ligand (CD154). Patients with the syndrome suffer from infections with opportunistic pathogens such as Cryptosporidium and Pneumocystis carinii. In this study, we demonstrate that activated T cells from patients with XHIM produce markedly reduced levels of IFN-gamma, fail to induce antigen-presenting cells to synthesize IL-12,...
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