Regulating complement in the kidney: insights from CFHR5 nephropathy

被引：24

作者：

Gale, Daniel P. ^{[1
]}

Pickering, Matthew C. ^{[2
]}

机构：

[1] Royal Free Hosp, UCL Ctr Nephrol, London NW3 2PF, England

[2] Univ London Imperial Coll Sci Technol & Med, Ctr Complement & Inflammat Res, London W12 0NN, England

来源：

DISEASE MODELS & MECHANISMS | 2011年 / 4卷 / 06期

基金：

英国惠康基金;

关键词：

HEMOLYTIC-UREMIC SYNDROME; DENSE DEPOSIT DISEASE; H-RELATED PROTEIN-5; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS; MACULAR DEGENERATION; C3; GLOMERULOPATHY; IMMUNE-RESPONSES; ACTIVATION; HEREDITARY; MUTATION;

D O I：

10.1242/dmm.008052

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of complement regulation that is endemic in Cyprus. The disease is characterised by haematuria, C3 glomerulonephritis and kidney failure. Its identification suggests a role for the CFHR5 protein in the regulation of complement in the kidney. In this review, we discuss how studying CFHR5 nephropathy can contribute to our understanding of the role of complement in kidney diseases such as dense deposit disease, C3 glomerulonephritis and atypical haemolytic uraemic syndrome.

引用

页码：721 / 726

页数：6

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