Activation of CVID patients' T cells with conventional antigens and superantigens.

Immunodeficiency Pub Date : 1993-01-01
M B Fischer, I Hauber, H Eggenbauer, V Thon, J Lokaj, H M Wolf, J W Mannhalter, M M Eibl
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Abstract

Defects in T cell function are known to be present in a subset of patients with CVID, but the true nature of these defects still has to be revealed. In prior studies we described that T cells from these patients show an impaired proliferative response following activation with recall antigens (E. coli, Tet. Tox., TBE and PPD). Gene expression of IL2 and IFN-gamma in patients' T cells following antigenic stimulation was significantly reduced compared to controls, while IL-2R transcripts were normal. To further characterize the defect we examined T cell responses to bacterial enterotoxins, collectively termed superantigens. Following stimulation with optimal (10 ng/ml p < 0.05) as well as suboptimal (1 ng/ml p < 0.0025) concentrations of staphylococcal enterotoxin A (SEA), proliferative response and cytokine release (IL-2 and IFNg) were significantly decreased in patients' T cells as compared to controls'. When patients' T cells were stimulated with staph. enterotox. C3 (SEC3) an even more pronounced difference between patients' and controls' T cells could be observed (10 ng/ml p < 0.002, 1 ng/ml p < 0.0005). Our data indicate that, in addition to the defect in antigen-induced T cell activation, T cells of CVID patients express a broader impairment in the interaction between the antigen presenting cell and the TCR.

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用常规抗原和超抗原激活CVID患者的T细胞。
已知T细胞功能缺陷存在于一小部分CVID患者中,但这些缺陷的真实性质仍有待揭示。在先前的研究中,我们描述了来自这些患者的T细胞在被召回抗原(大肠杆菌,Tet)激活后表现出受损的增殖反应。托克斯。(如:be和PPD)。与对照组相比,抗原刺激后患者T细胞中il -2和ifn - γ的基因表达显著降低,而IL-2R转录正常。为了进一步表征缺陷,我们检查了T细胞对细菌肠毒素的反应,统称为超级抗原。在给予最佳(10 ng/ml p < 0.05)和次优(1 ng/ml p < 0.0025)浓度的葡萄球菌肠毒素A (SEA)刺激后,与对照组相比,患者T细胞的增殖反应和细胞因子释放(IL-2和IFNg)显著降低。当病人的T细胞被葡萄球菌刺激时。enterotox。C3 (SEC3)在患者和对照组之间可以观察到更明显的差异(10 ng/ml p < 0.002, 1 ng/ml p < 0.0005)。我们的数据表明,除了抗原诱导的T细胞活化缺陷外,CVID患者的T细胞在抗原提呈细胞与TCR之间的相互作用中表现出更广泛的损伤。
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A PCR based X-chromosome inactivation assay for carrier detection in X-linked immunodeficiencies using differential methylation of the androgen receptor gene. Selection transduction defect (STD) due to Zap-70 kinase deficiency. A syndrome involving immunodeficiency and multiple intestinal atresias. Physical and genetic approaches to the isolation of the gene for X-linked agammaglobulinemia. Study of B and T cell phenotypes in blood from patients with common variable immunodeficiency (CVID).
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