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• W2131810356 abstract "X-linked lymphoproliferative disease (XLP) is an often-fatal immunodeficiency characterized by hypogammaglobulinemia, fulminant infectious mononucleosis, and/or lymphoma. The genetic lesion in XLP, SH2D1A, encodes the adaptor protein SAP (signaling lymphocytic activation molecule-associated [SLAM-associated] protein); however, the mechanism(s) by which mutations in SH2D1A causes hypogammaglobulinemia is unknown. Our analysis of 14 XLP patients revealed normal B cell development but a marked reduction in the number of memory B cells. The few memory cells detected were IgM(+), revealing deficient isotype switching in vivo. However, XLP B cells underwent proliferation and differentiation in vitro as efficiently as control B cells, which indicates that the block in differentiation in vivo is B cell extrinsic. This possibility is supported by the finding that XLP CD4(+) T cells did not efficiently differentiate into IL-10(+) effector cells or provide optimal B cell help in vitro. Importantly, the B cell help provided by SAP-deficient CD4(+) T cells was improved by provision of exogenous IL-10 or ectopic expression of SAP, which resulted in increased IL-10 production by T cells. XLP CD4(+) T cells also failed to efficiently upregulate expression of inducible costimulator (ICOS), a potent inducer of IL-10 production by CD4(+) T cells. Thus, insufficient IL-10 production may contribute to hypogammaglobulinemia in XLP. This finding suggests new strategies for treating this immunodeficiency." @default.
• W2131810356 created "2016-06-24" @default.
• W2131810356 creator A5006672507 @default.
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• W2131810356 date "2005-03-03" @default.
• W2131810356 modified "2023-10-02" @default.
• W2131810356 title "Impaired humoral immunity in X-linked lymphoproliferative disease is associated with defective IL-10 production by CD4+ T cells" @default.
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• W2131810356 doi "https://doi.org/10.1172/jci23139" @default.
• W2131810356 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/1059448" @default.
• W2131810356 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/15761493" @default.
• W2131810356 hasPublicationYear "2005" @default.
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