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中华肾病研究电子杂志 ›› 2025, Vol. 14 ›› Issue (04) : 214 -217. doi: 10.3877/cma.j.issn.2095-3216.2025.04.006

综述

补体H因子及其相关蛋白在IgA肾病中的作用
魏凯悦1, 宋一波2, 阎磊1, 曹慧霞1, 赵晓茹1, 邵凤民1,()   
  1. 1450000 郑州大学人民医院肾内科
    2014000 包头医学院第一附属医院
  • 收稿日期:2024-12-06 出版日期:2025-08-28
  • 通信作者: 邵凤民

Roles of complement H factor and its related proteins in IgA nephropathy

Kaiyue Wei1, Yibo Song2, Lei Yan1, Huixia Cao1, Xiaoru Zhao1, Fengmin Shao1,()   

  1. 1Department of Nephrology, People′s Hospital of Zhengzhou University, Zhengzhou 450000, Henan Province
    2First Affiliated Hospital of Baotou Medical College, Baotou 014000, Inner Mongolia Autonomous Region; China
  • Received:2024-12-06 Published:2025-08-28
  • Corresponding author: Fengmin Shao
引用本文:

魏凯悦, 宋一波, 阎磊, 曹慧霞, 赵晓茹, 邵凤民. 补体H因子及其相关蛋白在IgA肾病中的作用[J/OL]. 中华肾病研究电子杂志, 2025, 14(04): 214-217.

Kaiyue Wei, Yibo Song, Lei Yan, Huixia Cao, Xiaoru Zhao, Fengmin Shao. Roles of complement H factor and its related proteins in IgA nephropathy[J/OL]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2025, 14(04): 214-217.

IgA肾病是世界范围内最常见的原发性肾小球疾病,有20%~40%的IgA肾病患者最终进展成为终末期肾脏病。IgA肾病治疗尚无特异性药物,临床治疗以肾素血管紧张素-醛固酮系统抑制剂为基础,必要时加用类固醇皮质激素、免疫抑制剂。研究已经证实,补体激活的旁路途径是最常见的IgA肾病补体激活途径,而补体H因子及其相关蛋白是重要的旁路途径调节蛋白。随着研究的不断深入,越来越多的学者开始关注补体H因子在IgA肾病发病机制中的作用。本文系统综述了补体H因子及其相关蛋白在IgA肾病中的作用相关研究,为探究IgA肾病诊断及预后的生物标志物、开发补体抑制剂等靶向药物提供参考。

IgA nephropathy (IgAN) is the most common primary glomerular disease worldwide, with 20% to 40% of IgAN patients eventually progressing to end-stage renal disease. There is currently no specific drug for IgAN treatment, and its clinical treatment is based on renin-angiotensin aldosterone system inhibitors, with the addition of corticosteroid steroids and immunosuppressants if necessary. Research has confirmed that the alternative pathway for complement activation is the most common complement activation pathway in IgAN, and complement factor H and its related proteins are important regulatory proteins of the pathway. With the continuous deepening of research, more and more scholars have begun to pay attention to the role of complement H factor in the pathogenesis of IgAN. This article systematically reviewed the role of complement H factor and its related proteins in IgAN, providing reference for exploring biomarkers for IgAN diagnosis and prognosis, and developing targeted drugs such as complement inhibitors.

图1 H因子与H因子相关蛋白结构示意图[2]注:FHL:factor H-like protein,H因子样蛋白;FHR:H因子相关蛋白;H因子由20个补体控制蛋白结构域组成,N端补体控制蛋白结构域1~4具有补体调节活性,C端补体控制蛋白结构域19~20与其部分内部区域6~9作为表面识别位点
图2 CFHR3,1△保护IgA肾病肾脏受损机制图[11]注:CFHR3,1△:CFHRs基因的缺失;图左侧部分示IgA肾病损伤机制:补体激活循环血中的C3,促进含有C3的循环免疫复合物的形成,进而引起肾小球IgA和C3的沉积,损伤肾小球;图右侧部分示CFHR3,1△减轻IgA肾病损伤机制:CFHR1和CFHR3基因的缺失,血浆H因子相关蛋白1和3的水平降低,竞争性引起血清H因子水平升高,抑制补体的调节功能,减少对血浆C3的激活,阻碍含有C3的循环免疫复合物的形成,导致系膜细胞C3沉积减少,减轻对肾脏的损伤
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