Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity
| Autor: | Axel Bouchon, Marina Cella, Helen L. Grierson, Jennifer Lewis, Hideo Nakajima, Marco Colonna, Jeffrey I. Cohen, Colin S. Duckett |
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| Rok vydání: | 2000 |
| Předmět: |
Cytotoxicity
Immunologic Immunology Biology medicine.disease_cause Virus Cell Line Pathogenesis Antigens CD Signaling Lymphocytic Activation Molecule Family Cell surface receptor medicine Humans Immunology and Allergy Cytotoxic T cell Signaling Lymphocytic Activation Molecule Associated Protein Receptors Immunologic Receptor Membrane Glycoproteins Intracellular Signaling Peptides and Proteins X-linked lymphoproliferative disease medicine.disease Epstein–Barr virus Lymphoproliferative Disorders Killer Cells Natural Cancer research Signal transduction Carrier Proteins Signal Transduction |
| Zdroj: | European Journal of Immunology. 30:3309-3318 |
| ISSN: | 1521-4141 0014-2980 |
| DOI: | 10.1002/1521-4141(200011)30:11<3309::aid-immu3309>3.0.co;2-3 |
| Popis: | Patients with the X-linked lymphoproliferative disorder (XLPD) are unable to control Epstein-Barr virus (EBV)-induced infections and lymphoproliferation. This disease is caused by a deficit of SAP, an adapter protein involved in the signal transduction of several cell surface receptors of the CD2 superfamily. One of these receptors, called 2B4, is expressed on NK cells, cytotoxic T cells and myeloid cells and activates NK cell cytotoxicity. Here we show that XLPD patients have a defect of 2B4 receptor-mediated NK cell cytotoxicity. This defect may contribute to the pathogenesis of XLPD by reducing NK cell lysis of EBV-infected B cells. |
| Databáze: | OpenAIRE |
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