Roifman C M
Division of Immunology/Allergy, Hospital for Sick Children, Toronto, ON, Canada.
Immunodeficiency. 1995;5(3):193-211.
We have previously described a new type of selective T-cell deficiency (STD) characterized by persistent infections reminiscent of severe combined immunodeficiency (SCID). We show here that STD patients carry a mutation of zap-70 resulting in a loss of the activity of this kinase. The thymus of zap-70-/- patients shows the presence of CD4CD8 double positive cells in the cortex, however, only CD4 but not CD8 single positive cells are present in the medulla. Peripheral CD4+ T cells from the zap-70-/- exhibit markedly reduced tyrosine phosphorylation, fail to produce IL-2, and do not proliferate in response to TCR 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.
我们之前描述过一种新型的选择性T细胞缺陷（STD），其特征是持续感染，类似于严重联合免疫缺陷（SCID）。我们在此表明，STD患者携带zap - 70突变，导致该激酶活性丧失。zap - 70基因敲除患者的胸腺在皮质中显示存在CD4CD8双阳性细胞，然而，在髓质中仅存在CD4单阳性细胞，而不存在CD8单阳性细胞。来自zap - 70基因敲除患者的外周CD4 + T细胞酪氨酸磷酸化明显减少，无法产生白细胞介素-2，并且在有丝分裂原或抗原刺激TCR时不增殖。因此，Zap - 70激酶对于CD8单阳性T细胞的发育以及单阳性CD4 T细胞的信号转导和功能似乎是不可或缺的。
