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01-01-2007 | Editorial Commentary

MPGN II – genetically determined by defective complement regulation?

Authors: Christoph Licht, Ursula Schlötzer-Schrehardt, Michael Kirschfink, Peter F. Zipfel, Bernd Hoppe

Published in: Pediatric Nephrology | Issue 1/2007

Abstract

MPGN II is a rare disease which is characterized by complement containing deposits within the GBM. The disease is characterized by functional impairment of the GBM causing progressive loss of renal function eventually resulting in end stage renal disease.

It now becomes evident that in addition to C3NeF, which inhibits the inactivation of the alternative C3 convertase C3bBb, different genetically determined factors are also involved in the pathogenesis of MPGN II. These factors though different from C3NeF also result in defective complement regulation acting either through separate pathways or synergistically with C3NeF. Following the finding of MPGN II in Factor H deficient animals, patients with MPGN II were identified presenting with an activated complement system caused by Factor H deficiency. Factor H gene mutations result in a lack of plasma Factor H or in a functional defect of Factor H protein. Loss of Factor H function can also be caused by inactivating Factor H autoantibodies, C3 mutations preventing interaction between C3 and Factor H, or autoantibodies against C3.

Identification of patients with MPGN II caused by defective complement control may allow treatment by replacement of the missing factor via plasma infusion, thus possibly preventing or at least delaying disease progress.

Soluble: C1 inhibitor, C4 binding protein, Factor H and FHL-1 (Factor H like protein=alternate splice variant of Factor H); FHR-1 (Factor H related protein-1), Factor I, clusterin, vitronectin (S-protein) Membrane anchored: MCP (membrane cofactor protein); DAF (decay accelerating factor); CR1 (complement receptor 1)

Distribution of C3NeF in serum depending on different histological types of MPGN: MPGN II>MPGN I>MPGN III [24].

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Title: MPGN II – genetically determined by defective complement regulation?
Authors: Christoph Licht
Ursula Schlötzer-Schrehardt
Michael Kirschfink
Peter F. Zipfel
Bernd Hoppe
Publication date: 01-01-2007
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 1/2007
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-006-0299-8

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MPGN II – genetically determined by defective complement regulation?

Abstract

Keynote webinar | Spotlight on medication adherence

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Abstract

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