ArticleKidney international2023
Autoantibodies against laminin-521 are pathogenic in anti-glomerular basement membrane disease.
Article in Kidney international, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 15 citations in OpenAlex.
- Article
- Case Report: Anti-glomerular basement membrane disease during pregnancy with favorable renal outcome, sequential biopsies, and dual anti-α1/α3(IV) and anti-LM521 antibodies.Frontiers in immunology · 2026Article
- Immunological overlap stratification in anti-GBM disease: prognostic differences and serological correlations-a single-center retrospective cohort study.Frontiers in immunology · 2026Article
- Kidney-targeted nanoplatforms: Strategies and applications.Theranostics · 2026Review
- Elevated serum soluble α3(IV)NC1 correlates with kidney injury and worse outcome in patients with anti-glomerular basement membrane disease.Frontiers in immunology · 2026Article
- Article
- Perlecan is a novel target of autoantibodies in anti-glomerular basement membrane disease.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025Article
- The multiple autoantibodies in anti-GBM disease.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025Article
- Clinical and pathological features of patients with recurrent anti-glomerular basement membrane disease.Renal failure · 2025Review
- A rare case of sero-negative anti-glomerular basement membrane disease combined with diabetic nephropathy and literature review.Renal failure · 2025Review
- Basement membranes in lung development, disease, and repair.Matrix biology : journal of the International Society for Matrix Biology · 2025Review
- Kidney immunology from pathophysiology to clinical translation.Nature reviews. Immunology · 2025Review
- Bowman's capsule rupture and its clinical significance in patients with anti-glomerular basement membrane disease.Frontiers in immunology · 2025Article
- Case Report: Atypical anti-GBM nephritis coexisting with Henoch-Schönlein purpura nephritis: exploring the pathogenic nexus.Frontiers in immunology · 2025Article
- Complement in anti-glomerular basement membrane glomerulonephritis.Frontiers in immunology · 2025Review
- The fate of immune complexes in membranous nephropathy.Frontiers in immunology · 2024Review
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8 authors at 2 institutions in 3 countries.
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Abstract
Anti-glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disorder characterized by autoantibodies against GBM components. Evidence from human inherited kidney diseases and animal models suggests that the α, β, and γ chains of laminin-521 are all essential for maintaining the glomerular filtration barrier. We previously demonstrated that laminin-521 is a novel autoantigen within the GBM and that autoantibodies to laminin-521 are present in about one-third of patients. In the present study, we investigated the pathogenicity of autoantibodies against laminin-521 with clinical and animal studies. Herein, a rare case of anti-GBM disease was reported with circulating autoantibodies binding to laminin-521 but not to the NC1 domains of α1-α5(IV) collagen. Immunoblot identified circulating IgG from this patient bound laminin α5 and γ1 chains. A decrease in antibody levels was associated with improved clinical presentation after plasmapheresis and immunosuppressive treatments. Furthermore, immunization with laminin-521 in female Wistar-Kyoto rats induced crescentic glomerulonephritis with linear IgG deposits along the GBM, complement activation along with infiltration of T cells and macrophages. Lung hemorrhage occurred in 75.0% of the rats and was identified by the presence of erythrocyte infiltrates and hemosiderin-laden macrophages in the lung tissue. Sera and kidney-eluted antibodies from rats immunized with laminin-521 demonstrated specific IgG binding to laminin-521 but not to human α3(IV)NC1, while the opposite was observed in human α3(IV)NC1-immunized rats. Thus, our patient data and animal studies imply a possible independent pathogenic role of autoantibodies against laminin-521 in the development of anti-GBM disease.
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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.