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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 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 Citation Map 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 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. Related Collections Clinical Trials and Observations Immunobiology Phagocytes Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  IMMUNOBIOLOGY Functional characterization of natural killer cells in type I leukocyte adhesion deficiency Roberta Castriconi1, Alessandra Dondero1, Claudia Cantoni1,2,3, Mariella Della Chiesa1, Carola Prato1, Marina Nanni2, Maurilia Fiorini4, Lucia Notarangelo4, Silvia Parolini5, Lorenzo Moretta1,2,3, Luigi Notarangelo4, Alessandro Moretta1,3, and Cristina Bottino1,2 1 Dipartimento di Medicina Sperimentale, Università degli Studi di Genova, Genoa, Italy; 2 Istituto Giannina Gaslini, Genoa, Italy; 3 Centro di Eccellenza per le Ricerche Biomediche, Università degli Studi di Genova, Genoa, Italy; 4 Angelo Nocivelli Institute of Molecular Medicine, Department of Pediatrics, Università di Brescia, Italy; 5 Dipartimento di Scienze Biomediche e Biotecnologie, Università di Brescia, Italy In this study, we analyzed IL-2–activated polyclonal natural killer (NK) cells derived from 2 patients affected by leukocyte adhesion deficiency type I (LAD1), an immunodeficiency characterized by mutations of the gene coding for CD18, the β subunit shared by major leukocyte integrins. We show that LAD1 NK cells express normal levels of various triggering NK receptors (and coreceptors) 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 cocultured 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 monocyte-derived immature dendritic cells (DCs). Altogether our present study indicates that in LAD1 patients, 3 important functions of NK cells (eg, cytotoxicity, IFN- production, and DC editing) are only marginally affected and provides new insight on 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?

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