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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Human IL2RA null mutation mediates immunodeficiency with lymphoproliferation and autoimmunity
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Human IL2RA null mutation mediates immunodeficiency with lymphoproliferation and autoimmunity

机译：人IL2RA无效突变介导免疫缺陷和淋巴增殖和自身免疫

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摘要

Cell-surface CD25 expression is critical for maintaining immune function and homeostasis. As in few reported cases, CD25 deficiency manifests with severe autoimmune enteritis and viral infections. To dissect the underlying immunological mechanisms driving these symptoms, we analyzed the regulatory and effector T cell functions in a CD25 deficient patient harboring a novel IL2RA mutation. Pronounced lymphoproliferation, mainly of the CD8+ T cells, was detected together with an increase in T cell activation markers and elevated serum cytokines. However, Ag-specific responses were impaired in vivo and in vitro. Activated CD8+STAT5+ T cells with lytic potential infiltrated the skin, even though FOXP3+ Tregs were present and maintained a higher capacity to respond to IL-2 compared to other T-cell subsets. Thus, the complex pathogenesis of CD25 deficiency provides invaluable insight into the role of IL2/IL-2RA-dependent regulation in autoimmunity and inflammatory diseases.
机译：细胞表面CD25表达对于维持免疫功能和体内平衡至关重要。正如在少数报道的病例中一样，CD25缺乏症表现为严重的自身免疫性肠炎和病毒感染。为了剖析驱动这些症状的潜在免疫学机制，我们分析了具有新型IL2RA突变的CD25缺陷患者的调节性T细胞和效应T细胞功能。检测到明显的淋巴增殖，主要是CD8 + T细胞，以及T细胞活化标志物的增加和血清细胞因子的升高。但是，体内和体外银特异性反应受到损害。即使存在FOXP3 + Treg，并且与其他T细胞亚群相比，具有溶解潜能的活化CD8 + STAT5 + T细胞仍能渗入皮肤。因此，CD25缺乏症的复杂发病机理为IL2 / IL-2RA依赖性调节在自身免疫和炎性疾病中的作用提供了宝贵的见解。

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