在IgG4相关疾病中,与年龄相关的B细胞的扩张和表面制造者
Panpan Zhang1, Hui Lu2, Yu Peng3
1Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, State Key Laboratory of Complex Severe and Rare Diseases, Beijing; Department of Rheumatology and Immunology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
与年龄相关的B细胞 (ABCs) 在IgG4相关疾病 (IgG4-RD) 患者和小鼠模型中被扩展. 这些发现表明,ABCs在IgG4-RD的发病过程中起作用.
科学领域:
- 免疫学 免疫学 免疫学
- 细胞生物学 细胞生物学
背景情况:
- 免疫球蛋白G4相关疾病 (IgG4-RD) 是一种纤维炎症状况,具有复杂的发病因子.
- 与年龄相关的B细胞 (ABCs) 与各种自身免疫和炎症疾病有关.
研究的目的:
- 在患有IgG4-RD的患者中研究ABCs的表达和表面标记特征.
- 使用小鼠模型探索ABCs在IgG4-RD发病过程中的作用.
主要方法:
- 流细胞计和免疫光学用于分析IgG4-RD患者和健康对照者的循环和组织透的ABC.
- LatY136F敲入 (LAT) 鼠标模型,IgG4-RD的模型,被用于研究受影响器官中的ABC.
主要成果:
- 与健康对照组相比,未接受治疗的IgG4-RD患者的ABCs (CD19+CD21-T-bet+CD11c+) 显著增加,治疗后的数量下降.
- 现型分析揭示了IgG4-RD患者ABC上的明显的表面标记特征,包括上调的CD86,TACI和CD38.
- 在LAT小鼠的肺部中,ABCs也被发现增加,这表明它们参与了疾病病理学.
结论:
- 在IgG4-RD患者的外周血液和受影响组织中,ABC扩大.
- 在IgG4-RD小鼠模型中ABCs的存在表明它们在疾病中的潜在致病作用.
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