ArticleClinical journal of the American Society of Nephrology : CJASN2012
Causes of alternative pathway dysregulation in dense deposit disease.
Article in Clinical journal of the American Society of Nephrology : CJASN, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 102 papers.
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Who cites it
102 citing papers in PubMed, 181 citations in OpenAlex.
- Eculizumab for dense deposit disease and C3 glomerulonephritis.Clinical journal of the American Society of Nephrology : CJASN · 2012Trial
- Complement and kidney diseases: unlocking the opportunity of targeted treatments for glomerular diseases, including IgA nephropathy.Pediatric nephrology (Berlin, Germany) · 2026Review
- Characterizing Properdin-Inhibited C3 Nephritic Factors in Patients with Complement-Mediated Kidney Diseases.International journal of molecular sciences · 2026Article
- Long-term eculizumab treatment failure in C3 glomerulonephritis: two case reports.Clinical kidney journal · 2026Article
- Pregnancy-associated dense deposit disease with neurological complications caused by Salmonella Typhi: A case report.Qatar medical journal · 2026Article
- Acquired drivers of C3 glomerulopathy.Clinical kidney journal · 2025Review
- Functional Characterization of Anti-C3bBb Autoantibodies and C3 Glomerulopathy Phenotype.Journal of the American Society of Nephrology : JASN · 2025Article
- Monoclonal gammopathy-associated C3 glomerulonephritis secondary to follicular lymphoma: a case report.Frontiers in immunology · 2025Article
- C3 Glomerulopathy Recurs Early after Kidney Transplantation in Serial Biopsies Performed within the First 2 Years after Transplantation.Clinical journal of the American Society of Nephrology : CJASN · 2024Article
- Non-Invasive Biomarkers for Diagnosis, Risk Prediction, and Therapy Guidance of Glomerular Kidney Diseases: A Comprehensive Review.International journal of molecular sciences · 2024Review
- C3 Glomerulopathy: Novel Treatment Paradigms.Kidney international reports · 2024Review
- Defining Nephritic Factors as Diverse Drivers of Systemic Complement Dysregulation in C3 Glomerulopathy.Kidney international reports · 2024Article
- C3 glomerulopathy: a kidney disease mediated by alternative pathway deregulation.Frontiers in nephrology · 2024Review
- A Nef Precursor or Benign Counterpart: High Prevalence of Healthy Subjects with Antibody Reactivity to the C3-Convertase.Kidney360 · 2023Article
- Clinico-Pathogenic Similarities and Differences between Infection-Related Glomerulonephritis and C3 Glomerulopathy.International journal of molecular sciences · 2023Review
- A Monoclonal Antibody That Provides a Model for C3 Nephritic Factors.Monoclonal antibodies in immunodiagnosis and immunotherapy · 2023Article
- C3 glomerulopathies: dense deposit disease and C3 glomerulonephritis.Frontiers in medicine · 2023Review
- Kidney diseases.Immunological reviews · 2023Review
- C3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease.American journal of medical genetics. Part C, Seminars in medical genetics · 2022Review
- C3 Glomerulopathy: A Review with Emphasis on Ultrastructural Features.Glomerular diseases · 2022Review
42 more citing papers are in PubMed but not listed here.
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
Abstract
BACKGROUND AND
objectivesThis study was designed to investigate the causes of alternative pathway dysregulation in a cohort of patients with dense deposit disease (DDD). DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Thirty-two patients with biopsy-proven DDD underwent screening for C3 nephritic factors (C3Nefs), factor H autoantibodies (FHAAs), factor B autoantibodies (FBAAs), and genetic variants in CFH. C3Nefs were detected by: ELISA, C3 convertase surface assay (C3CSA), C3CSA with properdin (C3CSAP), two-dimensional immunoelectrophoresis (2DIEP), and immunofixation electrophoresis (IFE). FHAAs and FBAAs were detected by ELISA, and CFH variants were identified by Sanger sequencing.
resultsTwenty-five patients (78%) were positive for C3Nefs. Three C3Nef-positive patients were also positive for FBAAs and one of these patients additionally carried two novel missense variants in CFH. Of the seven C3Nef-negative patients, one patient was positive for FHAAs and two patients carried CFH variants that may be causally related to their DDD phenotype. C3CASP was the most sensitive C3Nef-detection assay. C3CASP and IFE are complementary because C3CSAP measures the stabilizing properties of C3Nefs, whereas IFE measures their expected consequence-breakdown of C3b.
conclusionsA test panel that includes C3CSAP, IFE, FHAAs, FBAAs, and genetic testing for CFH variants will identify a probable cause for alternative pathway dysregulation in approximately 90% of DDD patients. Dysregulation is most frequently due to C3Nefs, although some patients test positive for FHAAs, FBAAs, and CFH mutations. Defining the pathophysiology of DDD should facilitate the development of mechanism-directed therapies.
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