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- Title
Role of SLAM-Associated Protein in the Pathogenesis of Autoimmune Diseases and Immunological Disorders.
- Authors
Furukawa, Hiroshi; Tohma, Shigeto; Kitazawa, Hiroshi; Komori, Hiroaki; Nose, Masato; Ono, Masao
- Abstract
Signaling lymphocytic activation molecule (SLAM)-associated protein (SAP) is an adaptor molecule containing a Src homology 2 (SH2) domain. SAP is expressed in T cells and natural killer (NK) cells and binds to the cytoplasmic domains of SLAM family receptors, resulting in the subsequent recruitment of Fyn. The SAP ( SH2D1A) gene is located on the X chromosome and is responsible for X-linked lymphoproliferative disease, characterized by higher susceptibility to Epstein-Barr virus infection. The SAP-mediated signal is not only essential for the development of NKT cells, i.e. unconventional CD1d-restricted T cells with invariant Vα14 T cell receptors, but also for the regulation of the function of NK cells and conventional T cells. The role of SAP-mediated signaling in the induction of autoimmune diseases has been analyzed using animal models such as lupus, hepatitis, and graft-versus-host disease and is considered important in their pathogenesis in humans. In this review we highlight the current findings on SAP-mediated signaling in hematopoietic cells and discuss its importance in autoimmune diseases and immunological disorders.
- Publication
Archivum Immunologiae & Therapiae Experimentalis, 2010, Vol 58, Issue 1, p37
- ISSN
0004-069X
- Publication type
Article
- DOI
10.1007/s00005-009-0060-7