The SAP and SLAM families in immune responses and X-linked lymphoproliferative disease
- 1 October 2003
- journal article
- review article
- Published by Springer Science and Business Media LLC in Nature Reviews Immunology
- Vol. 3 (10), 813-821
- https://doi.org/10.1038/nri1202
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.Keywords
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