PMID: 8613493Nov 1, 1995Paper

A mutation in zap-70 protein tyrosine kinase results in a selective immunodeficiency

Journal of Clinical Immunology
C M Roifman

Abstract

We have previously described a new type of selective T-cell deficiency characterized by persistent infections reminiscent of severe combined immunodeficiency. We show here that selective T-cell deficiency patients carry a mutation of zap-70 protein tyrosine kinase, resulting in a loss of the activity of this kinase. The thymus of zap-70(-1-1) patients shows the presence of CD4CD8 double-positive cells in the cortex, however, only CD4, and not CD8, single-positive cells are present in the medulla. Peripheral CD4+ T cells from the zap-70(-1-1) 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 the signal transduction and function of single-positive CD4 T cells.

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Citations

Jun 11, 2005·Annals of Allergy, Asthma & Immunology : Official Publication of the American College of Allergy, Asthma, & Immunology·Francisco A BonillaUNKNOWN Joint Council of Allergy, Asthma and Immunology
Oct 7, 1997·The Journal of Experimental Medicine·M T CrowleyA L DeFranco
Jan 20, 2016·The Journal of Experimental Medicine·Alice Y ChanJennifer M Puck
Oct 21, 2009·Current Opinion in Allergy and Clinical Immunology·Pietro Luigi PolianiFabio Facchetti
Jan 6, 2001·The Journal of Immunology : Official Journal of the American Association of Immunologists·E Z TchilianP C Beverley
Jun 1, 1997·Clinical Immunology and Immunopathology·M A MusciG A Koretzky

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