Technologies

Summary

SAP (SLAM-associated protein) is a small lymphocyte-specific signaling molecule that is defective or absent in patients with XLP. SAP has unusually high affinity for SLAM (also called CD150) and has been suggested to function by blocking binding of SHP-2 or other SH2-containing signaling proteins to SLAM receptors. SAP has also been shown to be required for recruitment and activation of the Src-family kinase FynT after SLAM ligation, where the SAP SH2 domain binds to the SH3 domain of FynT and directly couples FynT to SLAM.

Mutations in the SH2D1 A gene on the Xq24-26 chromosome are known to be responsible for many cases of X-linked Lymphoproliferative syndrome.