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.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
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Review
MeSH terms
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Animals
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Antigens, CD
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Carrier Proteins / genetics
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Carrier Proteins / immunology*
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Glycoproteins / immunology*
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Humans
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Immunoglobulins / immunology*
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Intracellular Signaling Peptides and Proteins*
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Lymphoproliferative Disorders / genetics
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Lymphoproliferative Disorders / immunology*
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Models, Molecular
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Mutation
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Receptors, Cell Surface
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Signal Transduction / genetics
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Signal Transduction / immunology
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Signaling Lymphocytic Activation Molecule Associated Protein
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Signaling Lymphocytic Activation Molecule Family Member 1
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Transcription Factors / immunology
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src Homology Domains / genetics
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src Homology Domains / immunology
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src-Family Kinases / immunology
Substances
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Antigens, CD
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Carrier Proteins
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Glycoproteins
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Immunoglobulins
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Intracellular Signaling Peptides and Proteins
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Receptors, Cell Surface
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SH2D1A protein, human
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SH2D1B protein, human
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Signaling Lymphocytic Activation Molecule Associated Protein
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Transcription Factors
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Signaling Lymphocytic Activation Molecule Family Member 1
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src-Family Kinases