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  • Title: Defective T cell receptor signaling and CD8+ thymic selection in humans lacking zap-70 kinase.
    Author: Arpaia E, Shahar M, Dadi H, Cohen A, Roifman CM.
    Journal: Cell; 1994 Mar 11; 76(5):947-58. PubMed ID: 8124727.
    Abstract:
    We have previously described a type of selective T cell deficiency (STD) characterized by persistent infections reminiscent of severe combined immunodeficiency. We show here that STD patients carry a mutation of zap-70, resulting in loss of the activity of this kinase. The thymi of zap-70-/- patients show the presence of CD4+CD8+ cells in the cortex; however, only CD4, not CD8, single-positive cells are present in the medulla. Peripheral CD4+ T cells from the zap-70-/- patients exhibit markedly reduced tyrosine phosphorylation, fail to produce interleukin-2, and do not proliferate in response to T cell receptor stimulation by mitogens or antigens. Thus, Zap-70 kinase appears to be indispensable for the development of CD8 single-positive T cells as well as for signal transduction and function of single-positive CD4 T cells.
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