JOURNAL ARTICLE

The X-linked lymphoproliferative-disease gene product SAP regulates signals induced through the co-receptor SLAM

J Sayos, C Wu, M Morra, N Wang, X Zhang, D Allen, S van Schaik, L Notarangelo, R Geha, M G Roncarolo, H Oettgen, J E De Vries, G Aversa, C Terhorst
Nature 1998 October 1, 395 (6701): 462-9
9774102
In addition to triggering the activation of B- or T-cell antigen receptors, the binding of a ligand to its receptor at the cell surface can sometimes determine the physiological outcome of interactions between antigen-presenting cells, T and B lymphocytes. The protein SLAM (also known as CDw150), which is present on the surface of B and T cells, forms such a receptor-ligand pair as it is a self-ligand. We now show that a T-cell-specific, SLAM-associated protein (SAP), which contains an SH2 domain and a short tall, acts as an inhibitor by blocking recruitment of the SH2-domain-containing signal-transduction molecule SHP-2 to a docking site in the SLAM cytoplasmic region. The gene encoding SAP maps to the same area of the X chromosome as the locus for X-linked lymphoproliferative disease (XLP) and we found mutations in the SAP gene in three XLP patients. Absence of the inhibitor SAP in XLP patients affects T/B-cell interactions induced by SLAM, leading to an inability to control B-cell proliferation caused by Epstein-Barr virus infections.

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