ArticleThe American journal of pathology2002
The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type II: point mutations in the factor H coding sequence block protein secretion.
Article in The American journal of pathology, 2002. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 77 citations in OpenAlex.
- C3 glomerulopathy: a kidney disease mediated by alternative pathway deregulation.Frontiers in nephrology · 2024Review
- Renal diseases and the role of complement: Linking complement to immune effector pathways and therapeutics.Advances in immunology · 2021Review
- A case of C3 glomerulonephritis successfully treated with eculizumab.Pediatric nephrology (Berlin, Germany) · 2015Article
- Making sense of the spectrum of glomerular disease associated with complement dysregulation.Pediatric nephrology (Berlin, Germany) · 2014Review
- Is renal function associated with early age-related macular degeneration?Optometry and vision science : official publication of the American Academy of Optometry · 2014Article
- Dense deposit disease and C3 glomerulopathy.Seminars in nephrology · 2013Review
- Recent insights into C3 glomerulopathy.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2013Review
- Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies.Nature reviews. Nephrology · 2012Review
- Dense deposit disease.Molecular immunology · 2011Review
- Disease-associated N-terminal complement factor H mutations perturb cofactor and decay-accelerating activities.The Journal of biological chemistry · 2011Article
- Genetics and complement in atypical HUS.Pediatric nephrology (Berlin, Germany) · 2010Review
- Dense deposit disease and the factor H H402 allele.Clinical and experimental nephrology · 2008Article
- A critical analysis of disease-associated DNA polymorphisms in the genes of cattle, goat, sheep, and pig.Mammalian genome : official journal of the International Mammalian Genome Society · 2008Review
- Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice.The Journal of clinical investigation · 2008Article
- Translational mini-review series on complement factor H: renal diseases associated with complement factor H: novel insights from humans and animals.Clinical and experimental immunology · 2008Review
- Complement factor H: using atomic resolution structure to illuminate disease mechanisms.Advances in experimental medicine and biology · 2008Review
- New approaches to the treatment of dense deposit disease.Journal of the American Society of Nephrology : JASN · 2007Review
- MPGN II--genetically determined by defective complement regulation?Pediatric nephrology (Berlin, Germany) · 2007Article
- Pig complement regulator factor H: molecular cloning and functional characterization.Immunogenetics · 2003Article
Corrections and comments
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Authors and funding
5 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Porcine membranoproliferative glomerulonephritis type II in piglets of the Norwegian Yorkshire breed is considered the first animal model of human dense deposit disease. Porcine dense deposit disease is caused by the absence of the complement regulator factor H in plasma. Here we report the molecular basis for this absence. Single nucleotide exchanges at position C1590G and T3610G in the coding region of the factor H gene result in amino acid exchanges at nonframework residues L493V and I1166R that are located within SCR 9 and SCR 20, respectively. Apparently the L493V mutation represents a polymorphism whereas the I1166R causes the physiological consequences a block in protein secretion. Expression analysis shows comparable mRNA levels for factor H in liver tissue derived from both affected and healthy animals. In affected piglets, factor H protein is detected in increased amounts in liver cells. Factor H accumulates inside the hepatocytes and is not released as shown by Western blot analysis and immunohistochemistry. These data demonstrate that single amino acid exchanges of two nonframework amino acids either alone or in combination block protein secretion of factor H. This observation is also of interest for other human diseases in which factor H is involved, such as human factor H-associated form of hemolytic uremic syndrome.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.