缺陷的Treg生成和白细胞粘附缺陷中的3型免疫反应增加 1 1
Serife Erdem1, Sule Haskologlu2, Yesim Haliloglu1
1Erciyes University School of Medicine, Department of Medical Biology, Kayseri, Turkey; Erciyes University, Betul-Ziya Eren Genome and Stem Cell Center, Kayseri, Turkey.
Clinical immunology (Orlando, Fla.)
|July 11, 2023
概括
白细胞粘附性缺陷1型 (LAD-1) 患者表现出调控性T细胞 (Treg) 生成受损,T辅助细胞17 (Th17) 和先天性淋巴细胞3 (ILC3) 种群增加. 这些免疫细胞的变化表明3型曲免疫可能与LAD-1的自身免疫症状有关.
科学领域:
- 免疫学 免疫学 免疫学
- 遗传学 遗传学 是一个
- 细胞生物学 细胞生物学
背景情况:
- 白细胞粘附性缺陷1型 (LAD-1) 是一种由ITGB2基因突变引起的初级免疫缺陷.
- ITGB2突变会损害白细胞功能,导致复发性感染和炎症.
研究的目的:
- 研究致病性ITGB2突变对T助手17 (Th17) 和调控性T细胞 (Treg) 分化和功能的影响.
- 在LAD-1患者中分析先天性淋巴细胞 (ILC) 子集.
- 探索LAD-1中免疫细胞变化和自身免疫症状之间的关系.
主要方法:
- 来自LAD-1患者和健康对照的周围血液样本的分析.
- 在体外评估Treg和Th17细胞的分化和功能.
- 流式细胞计量以量化ILC子集.
- 测量血清细胞因子水平 (例如IL-23).
主要成果:
- 在LAD-1患者中,外周血液Treg细胞的百分比下降,Treg生成受损.
- 在LAD-1患者中观察到血清IL-23水平升高和PBMCs刺激后IL-17A产量增加.
- 在LAD-1患者中发现了CD18缺乏Th17细胞的更高百分比和ILC3亚组的显著升高.
- LAD-1 PBMCs在迁移,增殖和对亡的抗性增加方面表现出缺陷.
结论:
- 在LAD-1中ITGB2突变导致有缺陷的Treg生成,并促进Th17和ILC3扩张.
- 这些免疫失调表明LAD-1中存在3型扭曲免疫.
- 观察到的免疫特征可能有助于与LAD-1相关的自身免疫表现.
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