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Blood, 1 June 2007, Vol. 109, No. 11, pp. 4873-4881. Prepublished online as a Blood First Edition Paper on February 1, 2007; DOI 10.1182/blood-2006-08-038760. This Article Full Text (PDF) All Versions of this Article: blood-2006-08-038760v1 109/11/4873    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Castriconi, R. Articles by Bottino, C. Search for Related Content PubMed PubMed Citation Articles by Castriconi, R. Articles by Bottino, C. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted August 1, 2006 Accepted January 28, 2007 Functional characterization of natural killer cells in type I leukocyte adhesion deficiency Roberta Castriconi, Alessandra Dondero, Claudia Cantoni, Mariella Della Chiesa, Carola Prato, Marina Nanni, Maurilia Fiorini, Lucia Notarangelo, Silvia Parolini, Lorenzo Moretta, Luigi Notarangelo, Alessandro Moretta, and Cristina Bottino* Dipartimento di Medicina Sperimentale, Universita degli Studi di Genova, Genova, Italy Centro di Eccellenza per le Ricerche Biomediche, Universita degli Studi di Genova, Genova, Italy Istituto Giannina Gaslini, Genova, Italy Department of Pediatrics, Angelo Nocivelli Institute of Molecular Medicine, University of Brescia, Brescia, Italy Dipartimento di Scienze Biomediche e Biotecnologie, Universita di Brescia, Brescia, Italy * Corresponding author; email: cristina.bottino{at}unige.it. In this study we analyzed IL2-activated polyclonal NK cells derived from two patients affected by Leukocyte Adhesion Deficiency Type I, an immunodeficiency characterized by mutations of the gene coding for CD18, the subunit shared by major leukocyte integrins. We show that LAD1 NK cells express normal levels of various triggering NK receptors (and co-receptors) and that mAb-mediated engagement of these receptors results in the enhancement of both NK cytolytic activity and cytokine production. Moreover, these activating NK receptors were capable of recognizing their specific ligands on target cells. Thus, LAD1 NK cells similarly to normal NK cells were capable of killing most human tumor cells analyzed and produced high amounts of IFN when co-cultured in presence of target cells. Murine target cells represented a common exception, as they were poorly susceptible to LAD1 NK cells. Finally, LAD1 NK cells could efficiently kill or induce maturation of monocytes-derived immature dendritic cells. Altogether our present study indicates that in LAD1 patients, three important functions of NK cells (e.g. cytotoxicity, IFN- production and DC editing) are only marginally affected and provides new insight in the cooperation between activating receptors and LFA-1 in the induction of NK cell activation and function. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: Y. T. Bryceson, H.-G. Ljunggren, and E. O. Long Minimal requirement for induction of natural cytotoxicity and intersection of activation signals by inhibitory receptors Blood, September 24, 2009; 114(13): 2657 - 2666. [Abstract] [Full Text] [PDF] G. J. Jones, J. C. D. Wiseman, K. J. Marr, S. Wei, J. Y. Djeu, and C. H. Mody In contrast to anti-tumor activity, YT cell and primary NK cell cytotoxicity for Cryptococcus neoformans bypasses LFA-1 Int. Immunol., April 1, 2009; 21(4): 423 - 432. [Abstract] [Full Text] [PDF]

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