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肾脏病与透析肾移植杂志

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补体系统调节蛋白H因子家族异常与肾脏疾病

  

  • 出版日期:2020-10-28 发布日期:2020-10-30

Abnormal complement H factor family and kidney diseases

  • Online:2020-10-28 Published:2020-10-30

摘要: H因子(FH)家族是补体激活旁路途经的调节蛋白,非典型溶血性尿毒综合征、C3肾小球肾炎、致密物沉积病、IgA肾病、狼疮性肾炎等肾脏疾病均与之相关。遗传学研究提示FH家族在疾病的发生发展中具有重要作用。临床和功能学研究证实了基因突变导致的FH家族蛋白结构和浓度的改变影响补体旁路途径的激活。研究也促进了FH家族在疾病诊断与治疗上的应用。本文旨在对FH家族异常致肾脏疾病的研究进行综述并介绍其诊断与治疗上的进展,希望能为进一步的研究提供参考。

关键词: H因子, 肾脏疾病, 遗传学, 发病机制

Abstract: TThe H factor family is a regulatory protein for complement alternative pathway.The pathogenesis and progression of a variety of kidney diseases are related to H factor family,such as atypical hemolytic uremic syndrome,C3 glomerulonephritis,dense deposit disease,IgA nephropathy,lupus nephritis,etc.Genetic studies suggest that H factor family plays an important role in occurrence and development of some kidney diseases.Clinical and functional studies have confirmed that,structure and concentration of factor H family proteins affect the activation of alternative complement pathway.Studies also promote the application of factor H family in disease diagnosis and treatment.This article reviews the influence of factor H family on diseases and introduces the progress of diagnosis and treatment.