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Semin Immunol. 2004 Dec;16(6):409-19.

The SAP family of adaptors in immune regulation.

Author information

1
Unité INSERM U429, Hôpital Necker Enfants-Malades, 149 rue de Sèvres, 75015 Paris, France. latour@necker.fr

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.

PMID:
15541655
DOI:
10.1016/j.smim.2004.08.020
[Indexed for MEDLINE]

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