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T1  - Properdin binds independent of complement activation in an in vivo model of anti-glomerular basement membrane disease 
SN  -  /  
UR  - http://hdl.handle.net/10138/307703 
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A1  - 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 
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PB  -  
Y1  - 2018 
LA  - eng 
AB  - 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 n... 
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KW  - 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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