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Cutting Edge: Signaling Lymphocytic Activation Molecule-Associated Protein Controls NKT Cell Functions
- Source :
- The Journal of Immunology. 174:3153-3157
- Publication Year :
- 2005
- Publisher :
- The American Association of Immunologists, 2005.
-
Abstract
- X-linked lymphoproliferative disease (XLP) is a fatal immunological disorder that typically manifests following EBV infection. XLP patients exhibit a number of immune defects including abnormal T, B, and NK lymphocyte function. These defects have been attributed to mutations of Src homology 2 domain-containing gene 1A (SH2D1A), the gene encoding signaling lymphocytic activation molecule-associated protein (SAP), an intracellular adaptor molecule expressed in lymphocytes. We have observed that SAP knockout (SAPKO) mice and humans with XLP have a complete lack of CD1d-restricted NKT cells. As expected, SAPKO mice injected with the NKT cell agonist, α-galactosylceramide failed to generate NKT cell IFN-γ or IL-4. Furthermore, in contrast to wild-type littermates, SAPKO mice coinjected with OVA and α-galactosylceramide failed to mount OVA-specific CTL responses. These data suggest that an absence of NKT cells may underlie part of the immune dysregulation seen in SAPKO mice and in XLP patients.
Details
- ISSN :
- 15506606 and 00221767
- Volume :
- 174
- Database :
- OpenAIRE
- Journal :
- The Journal of Immunology
- Accession number :
- edsair.doi...........6d05ab16f5ae40e5cd275c942f30bdc1