التفاصيل البيبلوغرافية
| العنوان: |
The X-linked lymphoproliferative-disease gene product SAP regulates signals induced through the co-receptor SLAM |
| المؤلفون: |
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 |
| المصدر: |
Nature, Nature. 395(6701):462-469 |
| سنة النشر: |
1998 |
| الوصف: |
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. |
| نوع الوثيقة: |
redif-article |
| اللغة: |
English |
| DOI: |
10.1038/26683 |
| الإتاحة: |
https://ideas.repec.org/a/nat/nature/v395y1998i6701d10.1038_26683.html |
| رقم الأكسشن: |
edsrep.a.nat.nature.v395y1998i6701d10.1038.26683 |
| قاعدة البيانات: |
RePEc |
