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.

Original languageEnglish
Pages (from-to)3309-3318
Number of pages10
JournalEuropean Journal of Immunology
Issue number11
StatePublished - 2000


  • Epstein-Barr virus
  • Human
  • NK
  • SLAM-associated protein
  • X-linked lymphoproliferative


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