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 and C. Terhorst ()
Additional contact information
J. Sayos: Beth Israel Deaconess Medical Center, Harvard Medical School
C. Wu: Beth Israel Deaconess Medical Center, Harvard Medical School
M. Morra: Beth Israel Deaconess Medical Center, Harvard Medical School
N. Wang: Beth Israel Deaconess Medical Center, Harvard Medical School
X. Zhang: Beth Israel Deaconess Medical Center, Harvard Medical School
D. Allen: Beth Israel Deaconess Medical Center, Harvard Medical School
S. van Schaik: Beth Israel Deaconess Medical Center, Harvard Medical School
L. Notarangelo: University of Brescia
R. Geha: The Children's Hospital, Harvard Medical School
M. G. Roncarolo: Cellular Therapy Laboratory, Telethon Institute for Gene Therapy
H. Oettgen: The Children's Hospital, Harvard Medical School
J. E. De Vries: Novartis Forschungsinstitut Geselschaft mbH
G. Aversa: Novartis Forschungsinstitut Geselschaft mbH
C. Terhorst: Beth Israel Deaconess Medical Center, Harvard Medical School

Nature, 1998, vol. 395, issue 6701, 462-469

Abstract: Abstract 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 tail, 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.

Date: 1998
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DOI: 10.1038/26683

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