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Review
. 2009 Feb;24(2):385-7.
doi: 10.1093/ndt/gfn652. Epub 2008 Dec 4.

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 Zipfel  1 , Richard J H Smith, Christine Skerka
Affiliations
Review

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 Zipfel et al. Nephrol Dial Transplant. 2009 Feb.
No abstract available

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Figures

Fig. 1
Fig. 1
Alternative complement pathway activation occurs in the fluid phase and is controlled by Factor I and Factor H. In the absence of either Factor I or Factor H regulators, activation is uncontrolled and proceeds continuously resulting in the consumption of C3 and Factor B. In this situation, little active C3 is present that can deposit on biological surfaces. However, at biological surface either membrane-bound or surface-attached regulators like Factor H exist to control the further progression of complement activation. In the absence of Factor H or Factor H deficiency even the low-level C3 deposition is uncontrolled and complement activation occurs e.g. at the glomerular basement membrane, and results in the deposition of complement activation products. If Factor I is absent complement is uncontrolled in the fluid phase and consumed, however, the presence of Factor H restricts further low-level complement activation at the surface of the glomerular basement membrane.
See this image and copyright information in PMC

References

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    1. Pickering MC, Cook HT, Warren J, et al. Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H. Nat Genet. 2002;31:424–428. - PubMed
    1. Appel GB, Cook HT, Hageman G, et al. Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. J Am Soc Nephrol. 2005;6:1392–1403. - PubMed
    1. Smith RJ, Alexander J, Barlow PN, et al. Dense Deposit Disease Focus Group. New approaches to the treatment of dense deposit disease. J Am Soc Nephrol. 2007;18:2447–2456. - PMC - PubMed
    1. Zipfel PF, Mihlan M, Skerka C. The alternative pathway of complement: a pattern recognition system. Adv Exp Med Biol. 2007;598:80–92. - PubMed

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