Journal
ANNUAL REVIEW OF IMMUNOLOGY, VOL 29
Volume 29, Issue -, Pages 665-705Publisher
ANNUAL REVIEWS
DOI: 10.1146/annurev-immunol-030409-101302
Keywords
XLP; cytotoxicity; germinal centers; autoimmunity; innate lymphocytes
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Funding
- NATIONAL HUMAN GENOME RESEARCH INSTITUTE [ZIAHG000123] Funding Source: NIH RePORTER
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The signaling lymphocyte activation molecule (SLAM)-associated protein, SAP, was first identified as the protein affected in most cases of X-linked lymphoproliferative (XLP) syndrome, a rare genetic disorder characterized by abnormal responses to Epstein-Barr virus infection, lymphoproliferative syndromes, and dysgammaglobulinemia. SAP consists almost entirely of a single SH2 protein domain that interacts with the cytoplasmic tail of SLAM and related receptors, including 2B4, Ly108, CD84, Ly9, and potentially CRACC. SLAM family members are now recognized as important immunomodulatory receptors with roles in cytotoxicity, humoral immunity, autoimmunity, cell survival, lymphocyte development, and cell adhesion. In this review, we cover recent findings on the roles of SLAM family receptors and the SAP family of adaptors, with a focus on their regulation of the pathways involved in the pathogenesis of XLP and other immune disorders.
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