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. Although the acute
renal injury was likely due to acute neutrophil activation, we found properdin deposition
in C3-knockout mice that was not associated with IgG. Thus, properdin may deposit
in injured tissues in vivo independent of its main ligand C3.
Graphical abstract
Graphical Abstract
Keywords
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Article Info
Publication History
Published online: October 12, 2018
Accepted:
June 28,
2018
Received in revised form:
May 31,
2018
Received:
July 5,
2017
Identification
Copyright
© 2018 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.
