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NDT Advance Access originally published online on December 4, 2008
Nephrology Dialysis Transplantation 2009 24(2):385-387; doi:10.1093/ndt/gfn652
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© The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


Factor I and factor H deficiency in renal diseases: similar defects in the fluid phase have a different outcome at the surface of the glomerular basement membrane*

Peter F. Zipfel1,2, Richard J. H. Smith3,4 and Christine Skerka1

1 Department of Infection Biology, Leibniz Institute for Natural Product Research and Infection Biology, Hans Knöll Institute 2 Friedrich Schiller University, Jena, Germany 3 Department of Internal Medicine 4 Department of Otolaryngology, University of Iowa Carver College of Medicine, Iowa City, IA, USA

Correspondence and offprint requests to: Peter F. Zipfel, Department of Infection Biology, Leibniz Institute for Natural Product Research and Infection Biology, Hans Knöll Institute, Beutenbergstr. 11a, D-07745 Jena, Germany. Tel: +49-3641-532-13-00; Fax: +49-3641-532-0807; E-mail: peter.zipfel@hki-jena.de

Keywords: complement deficiency; Factor H; Factor I; MPGN

The first 10% of the full text of this article appears below.



   Summary of the key findings
 
Membranoproliferative glomerulonephritis type II (MPGN II), also termed dense deposit disease (DDD), is associated with dysregulation of the alternative pathway of complement in the circulation and on biosurfaces that leads to the local deposition of complement activation products along the glomerular basement membrane (GBM). As both Factor I and Factor H control alternative complement activation in a solution and as deficiency of Factor H leads to MPGN, Matthew Pickering and colleagues explored the possibility that the deficiency of Factor I might lead to a somewhat similar renal phenotype. They generated Factor I-deficient mice and compared the renal disease in these animals to that observed in Factor H-deficient mice and animals with combined Factor H and Factor I deficiency [1].

Renal disease in Factor H-deficient mice is characterized by C3 deposition on glomerular capillary walls, mesangial hypercellularity, peripheral capillary loop thickening and double . . . [Full Text of this Article]

Complement dysregulation—a cause for MPGN
What is in it for the practising nephrologist


   Take-home message
 

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