Clinical & Experimental Immunology · 1999 · 43 citations · 22 references
Life SciencesDermatologyImmunologyCell SignalingImmunopathologySignal TransductionAutoimmune DiseasesCell-mediated ImmunityImmunodeficiency DisordersImmunopathology Of SkinT Cell ActivationT Cell BiologyCd4 Positive T CellsCd4 T Cell ActivationCd8 T Cell ActivationPerivascular InflammationPrimary Immunodeficiency DisordersT-cell Immune ResponsesT-cell Mediated Skin DiseaseZap-70 Deficiency
CD8 deficiency is a rare primary immunodeficiency caused by the defect of a tyrosine kinase, ZAP-70, which transduces signals from the T cell receptor. We report here a case of CD8 deficiency, having CD4+ T cells with a unique phenotype. The patient's T cells did not respond to anti-CD3 stimulation in vitro, suggesting that they were naive. However, many CD4+ T cells with activated and memory phenotypes, which expressed CD45RO+, HLA-DR+ and CD25+, were present in the peripheral blood, and these cells accumulated in the perivascular area of his infiltrative erythematous skin lesions. The patient's T cells could be activated by a high concentration of phytohaemagglutinin (PHA), indicating the presence of an alternate signalling pathway which bypasses ZAP-70 and activates CD4+ T cells in vivo. The origin and role of activated CD4+ T cells in the pathogenesis involved in the skin lesions are discussed.
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S M Hsu, Laurence Raine, Herbert Fanger · Journal of Histochemistry & Cytochemistry · 1981 · 14.3K citations · Full text
ZAP-70: A 70 kd protein-tyrosine kinase that associates with the TCR ζ chain
Andrew C. Chan, Makio Iwashima, Christoph W. Turck et al. · Cell · 1992 · 1K citations
Defective T cell receptor signaling and CD8+ thymic selection in humans lacking Zap-70 kinase
Enrico Arpaia, Michal Shahar, Harjit Dadi et al. · Cell · 1994 · 554 citations