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Nat Rev Immunol. 2003 Oct;3(10):813-21.

The SAP and SLAM families in immune responses and X-linked lymphoproliferative disease.

Author information

1
Immunology Unit, Department of Cellular Biology and Pathology, Medical School, University of Barcelona, Barcelona 08036, Spain. engel@medicina.ub.es

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.

PMID:
14523387
DOI:
10.1038/nri1202
[Indexed for MEDLINE]

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