The DOI matches its Crossref record; 28 of the 28 references it cites were found in a registry or an index.
X-Linked Lymphoproliferative Disease
Open accessAbstract
2B4 is a surface molecule involved in activation of the natural killer (NK) cell–mediated cytotoxicity. It binds a protein termed Src homology 2 domain–containing protein (SH2D1A) or signaling lymphocyte activation molecule (SLAM)-associated protein (SAP), which in turn has been proposed to function as a regulator of the 2B4-associated signal transduction pathway. In this study, we analyzed patients with X-linked lymphoproliferative disease (XLP), a severe inherited immunodeficiency characterized by critical mutations in the SH2D1A gene and by the inability to control Epstein-Barr virus (EBV) infections. We show that, in these patients, 2B4 not only fails to transduce triggering signals, but also mediates a sharp inhibition of the NK-mediated cytolysis. Other receptors involved in NK cell triggering, including CD16, NKp46, NKp44, and NKp30, displayed a normal functional capability. However, their activating function was inhibited upon engagement of 2B4 molecules. CD48, the natural ligand of 2B4, is highly expressed on the surface of EBV+ B cell lines. Remarkably, NK cells from XLP patients could not kill EBV+ B cell lines. This failure was found to be the consequence of inhibitory signals generated by the interaction between 2B4 and CD48, as the antibody-mediated disruption of the 2B4–CD48 interaction restored lysis of EBV+ target cells lacking human histocompatibility leukocyte antigen (HLA) class I molecules. In the case of autologous or allogeneic (HLA class I+) EBV+ lymphoblastoid cell lines, restoration of lysis was achieved only by the simultaneous disruption of 2B4–CD48 and NK receptor–HLA class I interactions. Molecular analysis revealed that 2B4 molecules isolated from either XLP or normal NK cells were identical. As expected, in XLP-NK cells, 2B4 did not associate with SH2D1A, whereas similar to 2B4 molecules isolated from normal NK cells, it did associate with Src homology 2 domain–containing phosphatase 1.
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Self-citationp46, a Novel Natural Killer Cell–specific Surface Molecule That Mediates Cell Activation
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Lanier, L. L. (2005) · Annual Review of Immunology · doi:10.1146/annurev.immunol.23.021704.115526 · Cited by 2,769
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XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome
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Metadata
- Authors
- Parolini, S., Bottino, C., Falco, M., Augugliaro, R., Giliani, S., Franceschini, R., Ochs, H. D., Wolf, H., Bonnefoy, J.-Y., Biassoni, R., Moretta, L., Notarangelo, L. D., & Moretta, A.
- Venue
- The Journal of Experimental Medicine
- Publisher
- Rockefeller University Press
- Published
- 2000-07-31
- Volume
- 192
- Issue
- 3
- Pages
- 337-346
- ISSN
- 0022-1007 (ISSN-L)
- 1540-9538
- OpenAlex
- W2170419443
- Cited by
- 477
- References
- 28
- FWCI
- 13.05
- Type
- journal article
- Language
- en
- Open access
- green
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