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

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http://hdl.handle.net/10138/307703

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O'Flynn , J , Kotimaa , J , Faber-Krol , R , Koekkoek , K , Klar-Mohamad , N , Koudijs , A , Schwaeble , W J , Stover , C , Daha , M R & van Kooten , C 2018 , ' Properdin binds independent of complement activation in an in vivo model of anti-glomerular basement membrane disease ' , Kidney International , vol. 94 , no. 6 , pp. 1141-1150 . https://doi.org/10.1016/j.kint.2018.06.030

Title: Properdin binds independent of complement activation in an in vivo model of anti-glomerular basement membrane disease
Author: O'Flynn, Joseph; Kotimaa, Juha; Faber-Krol, Ria; Koekkoek, Karin; Klar-Mohamad, Ngaisah; Koudijs, Angela; Schwaeble, Wilhelm J.; Stover, Cordula; Daha, Mohamed R.; van Kooten, Cees
Contributor: University of Helsinki, University of Helsinki
Date: 2018-12
Language: eng
Number of pages: 10
Belongs to series: Kidney International
ISSN: 0085-2538
URI: http://hdl.handle.net/10138/307703
Abstract: 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.
Subject: alternative pathway
C1q
C3
C5b-9
classical pathway
complement
glomerulonephritis
inflammation glomerular basement membrane
properdin
ALTERNATIVE PATHWAY
FACTOR-H
INDUCED ARTHRITIS
C3B DEPOSITION
INJURY
MICE
ANTIBODY
CELLS
AMPLIFICATION
PROTECTION
3111 Biomedicine
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