title: SH2D1A and SLAM protein expression in human lymphocytes and derived cell lines.
abstract: The gene defect responsible for the X-linked lymphoproliferative disease (XLP) is associated with an impaired control of Epstein-Barr virus (EBV) infection. The gene has been recently identified and the encoded protein (designated SH2D1A, DSHP or SAP) was characterized. It is a 128 amino acid (aa) protein, containing a single Src homology 2 (SH2) domain. It interacts with signaling lymphocytic activation molecule (SLAM) expressed on the surface of activated T and B cells. We show that activated T, but not activated B, cells express the SH2D1A protein. NK cells express the protein as well. Tumor lines originating from B, T or NK cells exhibited similar SH2D1A protein expression as the corresponding normal cells, with some notable exceptions. EBV-carrying, tumor phenotype representative (type I), but not EBV-carrying lymphoblastoid cell line (LCL)-like (type III) or EBV-negative Burkitt lymphoma (BL) lines expressed SH2D1A. The phenotypic switch from type I to type III in the EBV-carrying BL line Mutu was associated with a down-regulation of SH2D1A and up-regulation of SLAM. In contrast to normal ex vivo and long-term activated NK cells, 2 of 3 NK leukemia lines expressed SLAM. All 3 lines expressed SH2D1A, like their normal counterparts.
authors.affiliation: Microbiology and Tumorbiology Center, Karolinska Institute, Stockholm, Sweden. Noemi.Nagy@mtc.ki.se
authors.forename: N
authors.initials: N
authors.lastname: Nagy
authors.forename: C
authors.initials: C
authors.lastname: Cerboni
authors.forename: K
authors.initials: K
authors.lastname: Mattsson
authors.forename: A
authors.initials: A
authors.lastname: Maeda
authors.forename: P
authors.initials: P
authors.lastname: Gogolák
authors.forename: J
authors.initials: J
authors.lastname: Sümegi
authors.forename: A
authors.initials: A
authors.lastname: Lányi
authors.forename: L
authors.initials: L
authors.lastname: Székely
authors.forename: E
authors.initials: E
authors.lastname: Carbone
authors.forename: G
authors.initials: G
authors.lastname: Klein
authors.forename: E
authors.initials: E
authors.lastname: Klein
mesh_terms: D000595:Amino Acid Sequence; D015703:Antigens, CD; D002352:Carrier Proteins / Q000032:analysis* / Q000097:blood; D002460:Cell Line; D006023:Glycoproteins / Q000032:analysis* / Q000097:blood; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis* / Q000097:blood; D047908:Intracellular Signaling Peptides and Proteins; D007694:Killer Cells, Natural / Q000737:chemistry; D007938:Leukemia / Q000378:metabolism*; D008214:Lymphocytes / Q000737:chemistry*; D008969:Molecular Sequence Data; D011956:Receptors, Cell Surface; D000071179:Signaling Lymphocytic Activation Molecule Associated Protein; D000071177:Signaling Lymphocytic Activation Molecule Family Member 1
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; D000071179:Signaling Lymphocytic Activation Molecule Associated Protein; D000071177:Signaling Lymphocytic Activation Molecule Family Member 1