Review
Nature Reviews Immunology 3, 813-821 (October 2003) | doi:10.1038/nri1202
The SAP and SLAM families in immune responses and X-linked lymphoproliferative disease
Pablo Engel1, Michael J. Eck2 & Cox Terhorst2 About the authors
Abstract
SAP (signalling lymphocytic activation molecule (SLAM)-associated protein) is a T- and natural killer (NK)-cell-specific protein containing a single SH2 domain encoded by a gene that is defective or absent in patients with X-linked lymphoproliferative syndrome (XLP). The SH2 domain of SAP binds with high affinity to the cytoplasmic tail of the haematopoietic cell-surface glycoprotein SLAM and five related receptors. SAP regulates signal transduction of the SLAM-family receptors by recruiting SRC kinases. Similarly, the SAP-related proteins EAT2A and EAT2B are thought to control signal transduction that is initiated by SLAM-related receptors in professional antigen-presenting cells. In this review, we discuss recent findings on the structure and function of proteins of the SAP and SLAM families.
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Author affiliations
- Immunology Unit, Department of Cellular Biology and Pathology, Medical School, University of Barcelona, Barcelona 08036, Spain.
- Department of Cancer Biology, Dana–Farber Cancer Institute, Harvard Medical School, Massachusetts 02215, USA.
- Department of Immunology, Beth Israel Deaconess Medical Center, Harvard Medical School, Massachusetts 02215, USA.
Correspondence to: Pablo Engel1 Email: engel@medicina.ub.es
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