Properdin binds independent of complement activation in an in vivo model of anti –glomerular basement membrane disease

Properdin is the only known positive regulator of complement activation by stabilizing the alternative pathway convertase through C3 binding, thus prolonging its half-life. Recent in  vitro studies suggest that properdin may act as a specific pattern recognition molecule. To better understand the role of properdin in vivo, we used an experimental model of acute anti-glomerular basement membrane disease with wild-type, C3- and properdin knockout mice. The model exhibited severe proteinuria, acute neutrophil infiltration and activation, classical and alternative pathway activation, and progressive glomerular deposition of properdin, C3 and C9.

Source: Kidney International - October 12, 2018 Category: Urology & Nephrology Authors: Joseph O ’Flynn, Juha Kotimaa, Ria Faber-Krol, Karin Koekkoek, Ngaisah Klar-Mohamad, Angela Koudijs, Wilhelm J. Schwaeble, Cordula Stover, Mohamed R. Daha, Cees van Kooten Tags: Basic Research Source Type: research