Review: Complement and its regulatory proteins in kidney diseases
Article first published online: 2 JUL 2010
© 2010 The Authors. Nephrology © 2010 Asian Pacific Society of Nephrology
Volume 15, Issue 7, pages 663–675, October 2010
How to Cite
LESHER, A. M. and SONG, W.-C. (2010), Review: Complement and its regulatory proteins in kidney diseases. Nephrology, 15: 663–675. doi: 10.1111/j.1440-1797.2010.01373.x
- Issue published online: 2 JUL 2010
- Article first published online: 2 JUL 2010
- Accepted manuscript online: 2 JUL 2010 12:00AM EST
- Accepted for publication 11 June 2010.Accepted manuscript online 2 July 2010.
- anti-GBM glomerulonephritis;
- atypical hemolytic uremic syndrome;
- complement regulatory proteins;
- ischemia-reperfusion injury;
- membranoproliferative glomerulonephritis type II
Complement is a part of the body's innate immune system that helps defend the host from microbial infection. It is tightly controlled by a number of cell surface and fluid-phase proteins so that under normal circumstances injury to autologous tissues is avoided. In many pathological settings, such as when the complement regulatory mechanisms are dysfunctional or overwhelmed, complement attack of autologous tissues can occur with severe, sometimes life-threatening consequences. The kidney appears to be particularly vulnerable to complement-mediated inflammatory injury and many kidney pathologies have been linked to abnormal complement activation. Clinical and experimental studies have shown that complement attack can be a primary cause in rare, genetically predisposed kidney diseases or a significant contributor to kidney injury caused by other etiological factors. Here we provide a brief review of recent advances on the activation and regulation of the complement system in kidney disease, with a particular emphasis on the relevance of complement regulatory proteins.