title: The SAP and SLAM families in immune responses and X-linked lymphoproliferative disease.
abstract: 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.
authors.affiliation: Immunology Unit, Department of Cellular Biology and Pathology, Medical School, University of Barcelona, Barcelona 08036, Spain. engel@medicina.ub.es
authors.forename: Pablo
authors.initials: P
authors.lastname: Engel
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Eck
authors.forename: Cox
authors.initials: C
authors.lastname: Terhorst
mesh_terms: D000818:Animals; D015703:Antigens, CD; D002352:Carrier Proteins / Q000235:genetics / Q000276:immunology*; D006023:Glycoproteins / Q000276:immunology*; D006801:Humans; D007136:Immunoglobulins / Q000276:immunology*; D047908:Intracellular Signaling Peptides and Proteins; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000276:immunology*; D008958:Models, Molecular; D009154:Mutation; D011956:Receptors, Cell Surface; D015398:Signal Transduction / Q000235:genetics / Q000276:immunology; D000071179:Signaling Lymphocytic Activation Molecule Associated Protein; D000071177:Signaling Lymphocytic Activation Molecule Family Member 1; D014157:Transcription Factors / Q000276:immunology; D018909:src Homology Domains / Q000235:genetics / Q000276:immunology; D019061:src-Family Kinases / Q000276:immunology
chemical_list: D015703:Antigens, CD; D002352:Carrier Proteins; D006023:Glycoproteins; D007136:Immunoglobulins; D047908:Intracellular Signaling Peptides and Proteins; D011956:Receptors, Cell Surface; C114953:SH2D1A protein, human; C103572:SH2D1B protein, human; D000071179:Signaling Lymphocytic Activation Molecule Associated Protein; D014157:Transcription Factors; D000071177:Signaling Lymphocytic Activation Molecule Family Member 1; D019061:src-Family Kinases