TH17 cells and regulatory T cells in primary immunodeficiency diseases.
TH17 cells and regulatory T cells in primary immunodeficiency diseases.
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DOI：
10.1016/j.jaci.2009.03.030
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发表时间：
2009-05
影响因子：
14.2
通讯作者：
Torgerson, Troy R.
中科院分区：
文献类型：
--
作者：
Ochs, Hans D.;Oukka, Mohamed;Torgerson, Troy R.
关键词：
在被独特的细胞因子激活后，CD 4+幼稚T细胞分化为辅助/效应（Th）和调节性T细胞（Treg）谱系，其特征在于不同的发育途径和独特的生物学功能。值得信赖的Th 1和Th 2二元系统已扩展到包括产生IL-17的Th 17细胞谱系，该细胞谱系在对感染性病原体的免疫反应和自身免疫性疾病的维持中发挥作用。作为一种平衡，TdR维持外周耐受并保护宿主免受自身攻击性淋巴细胞的侵害。Th 1细胞产生IFN-γ并参与细胞介导的免疫; Th 2细胞产生IL-4并有助于体液免疫; Th 17细胞产生IL-17并在对真菌和细胞外病原体的免疫应答中发挥重要作用; FOXP 3 + T细胞分泌TGF-β和IL-10并下调效应T细胞。常染色体显性遗传高IgE综合征是一种罕见的原发性免疫缺陷疾病，由STAT 3的亚纯型杂合突变引起，阻止了Th 17谱系分化，并增加了对念珠菌和念珠菌感染的易感性。FOXP 3突变干扰Treg发育并导致免疫失调、多内分泌病、X连锁肠病（IPEX）。导致Treg功能降低的其他单基因缺陷包括CD 25、STAT 5 b、AIRE和WASP。这些观察结果强调了功能不同的T细胞谱系在维持平衡的先天性和同源免疫系统中的重要性。
Following activation by unique cytokines, CD4+ naïve T cells differentiate into lineages of helper/effector (Th) and regulatory T cells (Treg) that are characterized by distinct developmental pathways and unique biological functions. The trusted binary system of Th1 and Th2 has been expanded to include the IL-17 producing Th17 cell lineage, which plays a role in immune responses to infectious agents and maintenance of autoimmune diseases. Acting as counterbalance, Tregs maintain peripheral tolerance and protect the host from autoaggressive lymphocytes. Th1 cells produce IFN-γ and are involved in cell-mediated immunity; Th2 cells produce IL-4 and contribute to humoral immunity; Th17 cells generate IL-17 and play an important role in immune responses to fungi and extracellular pathogens; FOXP3+ Tregs secrete TGF-β and IL-10 and downregulate effector T cells. Autosomal dominant hyper IgE syndrome, a rare Primary Immunodeficiency Disorder, is caused by hypomorphic heterozygous mutations of STAT3, preventing Th17 lineage differentiation, and increasing susceptibility to staphylococcus and Candida infections. Mutations in FOXP3 interfere with Treg development and cause Immune dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (IPEX). Other single gene defects resulting in reduced Treg function include CD25, STAT5b, AIRE, and WASP. These observations emphasize the importance of functionally distinct T cell lineages in maintaining a balanced innate and cognate immune system.
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