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。
The 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.
作者
铁丰红(综述)
于磊(审校)
TIE Fenghong;YU Lei(Department of Nephrology,Xing'an League people's hospital,Ulanhot 137400,China;Department of Nephrology,Inner Mongolia people's hospital,Hohhot 010017,China)
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2020年第5期464-468,共5页
Chinese Journal of Nephrology,Dialysis & Transplantation
基金
国家自然科学基金(81160087)
国家自然科学基金(816-60117)
内蒙古自治区自然科学基金(2018MS08008)
内蒙古自治区科技计划项目(201502107)
