ArticleJournal of clinical immunology2016
Testing the Activity of Complement Convertases in Serum/Plasma for Diagnosis of C4NeF-Mediated C3 Glomerulonephritis.
Article in Journal of clinical immunology, 2016. 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, 31 citations in OpenAlex.
- C3 glomerulopathy in children: experience at a resource-limited center.Clinical and experimental pediatrics · 2025Article
- Case Report: Possible C3 nephritic factor-driven complement-mediated severe hemolytic anemia and acute kidney injury in a child withFrontiers in immunology · 2025Article
- Defining Nephritic Factors as Diverse Drivers of Systemic Complement Dysregulation in C3 Glomerulopathy.Kidney international reports · 2024Article
- Genetic investigation of Nordic patients with complement-mediated kidney diseases.Frontiers in immunology · 2023Article
- Association between HLA alleles and sub-phenotype of childhood steroid-sensitive nephrotic syndrome.World journal of pediatrics : WJP · 2022Article
- Challenges in diagnostic testing of nephritic factors.Frontiers in immunology · 2022Review
- Complement Factor D (adipsin) Levels Are Elevated in Acquired Partial Lipodystrophy (Barraquer-Simons syndrome).International journal of molecular sciences · 2021Article
- Different Aspects of Classical Pathway Overactivation in Patients With C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative Glomerulonephritis.Frontiers in immunology · 2021Article
- Gain-of-Function Mutations R249C and S250C in Complement C2 Protein Increase C3 Deposition in the Presence of C-Reactive Protein.Frontiers in immunology · 2021Article
- The Immunopathology of Complement Proteins and Innate Immunity in Autoimmune Disease.Clinical reviews in allergy & immunology · 2020Review
- Validation of distinct pathogenic patterns in a cohort of membranoproliferative glomerulonephritis patients by cluster analysis.Clinical kidney journal · 2020Article
- C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy.Orphanet journal of rare diseases · 2019Article
- Complement activation products in the circulation and urine of primary membranous nephropathy.BMC nephrology · 2019Article
- Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy.Pediatric nephrology (Berlin, Germany) · 2019Review
- C3 glomerulopathy - understanding a rare complement-driven renal disease.Nature reviews. Nephrology · 2019Review
- Nephritic Factors: An Overview of Classification, Diagnostic Tools and Clinical Associations.Frontiers in immunology · 2019Review
- C3-Glomerulopathy Autoantibodies Mediate Distinct Effects on Complement C3- and C5-Convertases.Frontiers in immunology · 2019Article
- Factor B and C4b2a Autoantibodies in C3 Glomerulopathy.Frontiers in immunology · 2019Review
- C4 Nephritic Factors in C3 Glomerulopathy: A Case Series.American journal of kidney diseases : the official journal of the National Kidney Foundation · 2017Article
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Authors and funding
10 authors at 6 institutions in 3 countries.
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Abstract
Autoantibodies termed C3-nephritic factor (C3NeF), which stabilize convertases of the alternative complement pathway, often stimulate autoinflammatory diseases. However, knowledge about analogous autoantibodies acting on the classical pathway (C4NeF) is limited to a few reports, which indicate association with kidney dysfunction, systemic lupus erythematous, and infections. C4NeF may appear independently from C3NeF, but the lack of a routine diagnostic method predisposes C4NeF for being an underestimated player in autoinflammatory episodes. We tested the activity of classical convertases directly in serum/plasma to screen samples from 13 patients with C3 glomerulopathies and identified one patient showing significantly prolonged half-life of these enzymes. Observed effect was reproduced by immunoglobulins purified from patient's plasma and additionally confirmed on classical convertase built from purified components. Isolated immunoglobulins protected classical convertases from both spontaneous and inhibitor-driven decay but not from C4b proteolysis. The patient had a decreased serum level of C3, elevated sC5b-9, and normal concentrations of factor B and C4. Neither C3NeF nor other autoantibodies directed against alternative pathway proteins (factor H, factor B, factor I, C3, and properdin) were found. Genetic analysis showed no mutations in C3, CFB, CFH, CFI, MCP, THBD, and DGKE genes. Renal biopsy revealed a membranoproliferative pattern with intense C3 deposits. Our results underline the importance of C4NeF as an independent pathogenic factor and a need for the implementation of routine examination of classical convertase activity. Proposed method may enable robust inspection of such atypical cases.
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