ArticlePediatric nephrology (Berlin, Germany)2007
MPGN II--genetically determined by defective complement regulation?
Article in Pediatric nephrology (Berlin, Germany), 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 39 citations in OpenAlex.
- A clinical approach to children with C3 glomerulopathy.Pediatric nephrology (Berlin, Germany) · 2022Review
- Long-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children.Kidney international reports · 2020Article
- [Membranoproliferative glomerulonephritis and C3 glomerulopathy].Der Internist · 2019Review
- Membranoproliferative glomerulonephritis and C3 glomerulopathy in children: change in treatment modality? A report of a case series.Clinical kidney journal · 2018Article
- Viral-derived complement inhibitors: current status and potential role in immunomodulation.Experimental biology and medicine (Maywood, N.J.) · 2017Review
- American Society of Nephrology clinical pathological conference.Clinical journal of the American Society of Nephrology : CJASN · 2014Article
- Trends in pediatric primary membranoproliferative glomerulonephritis costs and complications.Pediatric nephrology (Berlin, Germany) · 2012Article
- Kidney diseases caused by complement dysregulation: acquired, inherited, and still more to come.Clinical & developmental immunology · 2012Review
- Dense deposit disease associated with monoclonal gammopathy of undetermined significance.American journal of kidney diseases : the official journal of the National Kidney Foundation · 2010Article
- Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway.Kidney international · 2009Article
- A microstructural retinal analysis of membrano-proliferative glomerulonephritis type II.The British journal of ophthalmology · 2008Article
- Prognostic factors in children with membranoproliferative glomerulonephritis type I.Pediatric nephrology (Berlin, Germany) · 2008Article
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Authors and funding
5 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
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.
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