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

Nat Rev Immunol. 2003 Oct;3(10):813-21. doi: 10.1038/nri1202.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Animals
  • Antigens, CD
  • Carrier Proteins / genetics
  • Carrier Proteins / immunology*
  • Glycoproteins / immunology*
  • Humans
  • Immunoglobulins / immunology*
  • Intracellular Signaling Peptides and Proteins*
  • Lymphoproliferative Disorders / genetics
  • Lymphoproliferative Disorders / immunology*
  • Models, Molecular
  • Mutation
  • Receptors, Cell Surface
  • Signal Transduction / genetics
  • Signal Transduction / immunology
  • Signaling Lymphocytic Activation Molecule Associated Protein
  • Signaling Lymphocytic Activation Molecule Family Member 1
  • Transcription Factors / immunology
  • src Homology Domains / genetics
  • src Homology Domains / immunology
  • src-Family Kinases / immunology


  • Antigens, CD
  • Carrier Proteins
  • Glycoproteins
  • Immunoglobulins
  • Intracellular Signaling Peptides and Proteins
  • Receptors, Cell Surface
  • SH2D1A protein, human
  • SH2D1B protein, human
  • Signaling Lymphocytic Activation Molecule Associated Protein
  • Transcription Factors
  • Signaling Lymphocytic Activation Molecule Family Member 1
  • src-Family Kinases