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J. Exp. Med.,
Volume 187, Number 1, January 5, 1998 117-122
By

From the * Laboratoire d'Histocompatibilité, Contrat Jeune Formation Institut National de la Santé et
de la Recherche Médicale 94-03, and Institut National de la Santé et de la Recherche Médicale
U.311, Etablissement de Transfusion Sanguine, Strasbourg 67065, France; In this paper we describe the function and phenotype of natural killer (NK) lymphocytes from
HLA class I-deficient patients. These cells are, as has been previously reported, unable to lyse HLA class I
Service de Pédiatrie,
Hôpital de Hautepierre, Strasbourg 67098, France; and § Istituto di Istologia ed Embriologia Generale,
Università di Genova, 16132 Genova, Italy and Dipartimento di Scienze Biomediche e
Biotechnologie, Università di Brescia, 25100 Brescia, Italy
K562 cells, but are able to perform antibody-dependent cellular cytotoxicity
(ADCC), although with lower efficiency as compared to NK cells from normal individuals.
Transporter associated to antigen processing (TAP)
NK cells proliferate when cultured in the
presence of lymphoblastoid B cells (B-LCs) and interleukin 2 and develop a spectrum of cytotoxicity similar to that of activated normal NK cells. Importantly, activation of the TAP
NK
cells induces strong cytotoxicity to autologous B-LCs. Analysis of the phenotype of circulating
TAP
NK lymphocytes showed them to display a normal diverse repertoire of HLA class I-specific
NK receptors. These receptors were expressed at normal levels, apart from the CD94-NKG2A
complex, which appeared to be overexpressed. This latter finding could reflect an adaptation to
the low expression of HLA class I molecules. Finally, functional analyses indicated that the inhibitory receptors in TAP
individuals can transduce inhibitory signals. Our results suggest that
in vivo, the NK cells of TAP
patients could participate in immune defense, at least through ADCC, but upon activation, may be involved in autoimmune processes.
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