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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Intact T cell responses in ataxia telangiectasia.
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Intact T cell responses in ataxia telangiectasia.

机译：共济失调毛细血管扩张的完整T细胞反应。

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摘要

Ataxia telangiectasia (A-T) is an autosomal recessive multisystem disorder associated with a variable immune deficiency. The mechanism for this remains unclear. Qualitative and quantitative defects of cellular immunity have been previously reported. However, despite laboratory evidence of significant immune abnormalities, opportunistic infections are uncommon. To address this discrepancy, we analyzed cytokine production by quantitative real-time PCR and T cell function at the single cell level by flow cytometry in four A-T patients. CD4 and CD8 T cell subsets from these patients displayed intact signaling in response to anti-CD3 stimulation, similar to controls. Stimulated T cells from A-T patients also produced normal to increased levels of Th1 (IL-2, IFN-gamma) and Th2 (IL-10, IL-4) cytokines, relative to control values. Our results suggest that T cells from A-T patients may be more functionally intact than previously observed. This helps to explain the paucity of opportunistic infections encountered, unlike that encountered in other primary immunodeficiencies.
机译：共济失调毛细血管扩张症（A-T）是与可变的免疫缺陷相关的常染色体隐性多系统疾病。其机制仍不清楚。先前已经报道了细胞免疫的定性和定量缺陷。然而，尽管实验室证据显示存在明显的免疫异常，但机会性感染并不常见。为了解决这一差异，我们通过定量实时PCR和流式细胞术在四名A-T患者中通过单细胞水平的T细胞功能分析了细胞因子的产生。这些患者的CD4和CD8 T细胞亚群在响应抗CD3刺激后表现出完整的信号传导，类似于对照组。相对于对照值，来自A-T患者的受激T细胞产生的Th1（IL-2，IFN-γ）和Th2（IL-10，IL-4）细胞因子水平正常至升高。我们的结果表明，来自A-T患者的T细胞可能比以前观察到的功能更完整。这有助于解释所遇到的机会性感染很少，这与其他原发性免疫缺陷所遇到的机会感染不同。

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