Department of Immune Regulation, Tokyo Medical and Dental University, Tokyo, Japan.
Ann N Y Acad Sci. 2011 Dec;1246:34-40. doi: 10.1111/j.1749-6632.2011.06280.x.
Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by atopic dermatitis associated with extremely high serum IgE levels and susceptibility to staphylococcal skin abscesses and pneumonia. Recent studies have identified dominant negative mutations in the signal transducer and activator of transcription 3 gene (STAT3) as a major molecular cause of classical hyper-IgE syndrome, but the molecular mechanisms underlying this syndrome remain unclear. We recently showed that the impaired development of interleukin 17 (IL-17)-producing T helper cells (Th17 cells) due to defective IL-6 and IL-23 signaling in T cells, and the impaired generation of induced regulatory T (iT(reg) ) cells from defective IL-10 signaling in dendritic cells, may account for the immunological abnormalities of hyper-IgE syndrome. These findings open up possibilities for exploring new approaches to the treatment of HIES patients.
高免疫球蛋白 E 综合征（HIES）是一种以特应性皮炎为特征的原发性免疫缺陷病，伴有极高的血清 IgE 水平和对葡萄球菌皮肤脓肿和肺炎的易感性。最近的研究已经确定信号转导和转录激活因子 3 基因（STAT3）的显性负突变是经典高免疫球蛋白 E 综合征的主要分子原因，但该综合征的分子机制尚不清楚。我们最近表明，由于 T 细胞中 IL-6 和 IL-23 信号转导缺陷导致白细胞介素 17（IL-17）产生的辅助性 T 细胞（Th17 细胞）发育受损，以及由于树突状细胞中 IL-10 信号转导缺陷导致诱导性调节 T（iTreg）细胞生成受损，可能导致高免疫球蛋白 E 综合征的免疫异常。这些发现为探索治疗 HIES 患者的新方法提供了可能性。
