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NFKB2 regulates human Tfh and Tfr pool formation and germinal center potential.
Clinical Immunology ( IF 4.5 ) Pub Date : 2019-11-18 , DOI: 10.1016/j.clim.2019.108309 Pasqualina De Leo 1 , Luisa Gazzurelli 2 , Manuela Baronio 2 , Davide Montin 3 , Silvia Di Cesare 4 , Carmen Giancotta 4 , Francesco Licciardi 3 , Caterina Cancrini 4 , Alessandro Aiuti 5 , Alessandro Plebani 2 , Maria Pia Cicalese 6 , Vassilios Lougaris 2 , Georgia Fousteri 1
Clinical Immunology ( IF 4.5 ) Pub Date : 2019-11-18 , DOI: 10.1016/j.clim.2019.108309 Pasqualina De Leo 1 , Luisa Gazzurelli 2 , Manuela Baronio 2 , Davide Montin 3 , Silvia Di Cesare 4 , Carmen Giancotta 4 , Francesco Licciardi 3 , Caterina Cancrini 4 , Alessandro Aiuti 5 , Alessandro Plebani 2 , Maria Pia Cicalese 6 , Vassilios Lougaris 2 , Georgia Fousteri 1
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Mutations affecting the non-canonical pathway of NF-κB were recently identified to underlie a form of common variable immunodeficiency strongly associated with autoimmunity. Although intrinsic B-cell abnormalities explain most of the humoral defects of this disease, detailed data on the impact of NFKB2 on follicular helper (Tfh) and regulatory (Tregs) T cells are scarce. Here, we show that Tfh, CXCR5+, and CXCR5- Treg cell subsets were significantly reduced in patients heterozygous for a truncating mutation of NFKB2. Plasma CXCL13 levels were reduced, underlining an important role for NFKB2 in regulating the germinal center (GC) response. Proinflammatory IFNγ, IL-17 and IL-10 cytokine production by CD4 T cells was lower in the mutated patients, but the production of IL-4 and IL-21 was not altered. Taken together, our findings show that NFKB2 influences the quality and efficiency of human GC reaction, by affecting not only the B cells but also GC-relevant T cell subsets.
中文翻译：
NFKB2调节人类Tfh和Tfr库的形成以及生发中心的潜力。
最近发现影响NF-κB非经典途径的突变是与自身免疫密切相关的常见可变免疫缺陷形式的基础。尽管内在的B细胞异常可以解释这种疾病的大多数体液缺陷，但是关于NFKB2对卵泡辅助性（Tfh）和调节性（Tregs）T细胞影响的详细数据却很少。在这里，我们显示杂合子的NFKB2截短突变患者的Tfh，CXCR5 +和CXCR5-Treg细胞亚群显着降低。血浆CXCL13水平降低，突显了NFKB2在调节生发中心（GC）反应中的重要作用。在突变的患者中，CD4 T细胞产生的促炎性IFNγ，IL-17和IL-10细胞因子较低，但IL-4和IL-21的产生没有改变。在一起
更新日期：2019-11-18
中文翻译：
NFKB2调节人类Tfh和Tfr库的形成以及生发中心的潜力。
最近发现影响NF-κB非经典途径的突变是与自身免疫密切相关的常见可变免疫缺陷形式的基础。尽管内在的B细胞异常可以解释这种疾病的大多数体液缺陷，但是关于NFKB2对卵泡辅助性（Tfh）和调节性（Tregs）T细胞影响的详细数据却很少。在这里，我们显示杂合子的NFKB2截短突变患者的Tfh，CXCR5 +和CXCR5-Treg细胞亚群显着降低。血浆CXCL13水平降低，突显了NFKB2在调节生发中心（GC）反应中的重要作用。在突变的患者中，CD4 T细胞产生的促炎性IFNγ，IL-17和IL-10细胞因子较低，但IL-4和IL-21的产生没有改变。在一起
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