The SAP family of adaptors in immune regulation

Semin Immunol. 2004 Dec;16(6):409-19. doi: 10.1016/j.smim.2004.08.020.

Abstract

SAP and EAT-2 define a new class of adaptor proteins composed almost exclusively of a Src homology 2 (SH2) domain. By way of their SH2 domain, SAP-like adaptors interact with tyrosine-based motifs in the cytoplasmic region of SLAM-related receptors, a family of immune cell-specific molecules involved in immunoregulation. Recent findings indicate that SAP is required for the functions of SLAM family receptors, as a consequence of its ability to promote recruitment of Src-related protein tyrosine kinase FynT and allow SLAM-related receptors to transduce tyrosine phosphorylation signals. SAP is mutated in X-linked lymphoproliferative (XLP) syndrome, a rare inherited human disease characterized by an deregulated immune response to Epstein-Barr virus infection. Several lines of evidence indicate that defects in the activities of SLAM-related receptors caused by SAP deficiency account for the immune dysfunctions associated with XLP.

Publication types

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

MeSH terms

  • Adaptor Proteins, Signal Transducing / classification
  • Adaptor Proteins, Signal Transducing / metabolism*
  • Animals
  • Humans
  • Immunity / immunology*
  • Lymphocytes / cytology
  • Lymphocytes / immunology
  • Signal Transduction
  • Syndrome

Substances

  • Adaptor Proteins, Signal Transducing