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Published on: March 17, 2014
Altered lymphocyte responses and cytokine production in mice deficient in the X-linked lymphoproliferative disease
M J Czar1, E N Kersh, L A Mijares
1National Human Genome Research Institute, National Cancer Institute, and National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, MD 20892, USA.
Abstract:
We have introduced a targeted mutation in SH2D1A/DSHP/SAP, the gene responsible for the human genetic disorder X-linked lymphoproliferative disease (XLP). SLAM-associated protein (SAP)-deficient mice had normal lymphocyte development, but on challenge with infectious agents, recapitulated features of XLP. Infection of SAP- mice with lymphocyte choriomeningitis virus (LCMV) or Toxoplasma gondii was associated with increased T cell activation and IFN-gamma production, as well as a reduction of Ig-secreting cells. Anti-CD3-stimulated splenocytes from uninfected SAP- mice produced increased IFN-gamma and decreased IL-4, findings supported by decreased serum IgE levels in vivo. The Th1 skewing of these animals suggests that cytokine misregulation may contribute to phenotypes associated with mutation of SH2D1A/SAP.
