Absence of ZAP-70 prevents signaling through the antigen receptor on peripheral blood T cells but not on thymocytes.

摘要: Recently, a severe combined immunodeficiency syndrome with deficiency of CD8+ peripheral T cells and TCR signal transduction defect in CD4+ was associated mutations ZAP-70. Since signaling is required developmental decisions resulting mature CD4 (and CD8) cells, the presence expressing TCRs incapable these patients paradoxical. Here, we show that on thymocytes, but not from ZAP-70-deficient patient are capable signaling. Moreover, thymocyte line derived this can signal, homologous kinase Syk present at high levels tyrosine phosphorylated after stimulation. Thus, may compensate for loss ZAP-70 account thymic selection least subset (CD4+) patients.