| First Author | Chung B | Year | 2005 |
| Journal | J Immunol | Volume | 174 |
| Issue | 6 | Pages | 3153-7 |
| PubMed ID | 15749842 | Mgi Jnum | J:97690 |
| Mgi Id | MGI:3576142 | Doi | 10.4049/jimmunol.174.6.3153 |
| Citation | Chung B, et al. (2005) Signaling lymphocytic activation molecule-associated protein controls NKT cell functions. J Immunol 174(6):3153-7 |
| abstractText | 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, alpha-galactosylceramide failed to generate NKT cell IFN-gamma or IL-4. Furthermore, in contrast to wild-type littermates, SAPKO mice coinjected with OVA and alpha-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. |
