ArticleJournal of the American Society of Nephrology : JASN2019
Exostosin 1/Exostosin 2-Associated Membranous Nephropathy.
Article in Journal of the American Society of Nephrology : JASN, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 164 papers, 2 of them syntheses that pooled it.
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Who cites it
164 citing papers in PubMed, 2 syntheses or guidelines pooled it, 296 citations in OpenAlex.
- PLA2R autoantibodies, a multifaceted biomarker in nephrotic syndrome and membranous nephropathy.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025Pooled it
- Secondary Membranous Nephropathy. A Narrative Review.Frontiers in medicine · 2020Pooled it
- Lipoic acid supplementation associated with neural epidermal growth factor-like 1 (NELL1)-associated membranous nephropathy.Kidney international · 2021Trial
- MHC class II and PLA2R investigating the epitope presentation deficits driving antibody production in membranous nephropathy.International urology and nephrology · 2026Review
- MPO-ANCA-associated glomerulonephritis with immune complex deposition and membranous features in a patient with Sjögren's syndrome: a case report.CEN case reports · 2026Article
- The Synthetic Melanocortin Agonist NDP-MSH Ameliorates THSD7A-Associated Membranous Nephropathy in an Active Immunization Mouse Model.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2026Article
- Nephrin autoimmunity: signal, noise, and a path to clarity.The Journal of clinical investigation · 2026Article
- Optimization of a Target Antigen Diagnostic Pathway for Membranous Nephropathy.Kidney international reports · 2026Article
- Multiplexed Detection of Membranous Nephropathy Antigens by Multi-Reaction Monitoring Mass Spectrometry.Kidney international reports · 2026Article
- Complement Profiling in Glomerular Disease: Insights from Laser Microdissection and Mass Spectrometry.Clinical journal of the American Society of Nephrology : CJASN · 2026Review
- Detection of Exostosin 1 in Lupus Nephritis: Prevalence, Clinico-Pathologic and Renal Outcome Correlations.Diagnostics (Basel, Switzerland) · 2026Article
- Genetic Testing and Kidney Biopsies: Complementary to Another, not Substitutive.Kidney international reports · 2026Article
- Application of the nephrotoxic serum nephritis model in glomerulonephritis research.Molecular medicine (Cambridge, Mass.) · 2026Review
- Proteomic Profiling of Complement Components in Glomerular Disease.Laboratory investigation; a journal of technical methods and pathology · 2026Article
- Glycoinformatic Profiling of Label-Free Intact Heparan Sulfate Oligosaccharides.Molecular & cellular proteomics : MCP · 2026Article
- Membranous Nephropathy: Antigenic Landscape and a Novel Pathogenetic Model.International journal of molecular sciences · 2026Review
- Protocadherin FAT1-associated membranous nephropathy after hematopoietic stem cell transplantation-a cohort study with clinico-pathological correlations.Clinical kidney journal · 2026Article
- Circulating Testican-2 and MGT5A are Markers of Membranous Nephropathy.Kidney international reports · 2026Article
- Liver-kidney crosstalk in primary biliary cholangitis: insights into the mechanisms of renal involvement.Frontiers in immunology · 2026Review
- Contemporary review of primary membranous nephropathy.Frontiers in immunology · 2026Review
104 more citing papers are in PubMed but not listed here.
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Authors and funding
10 authors at 3 institutions in 2 countries.
Funding
Abstract
backgroundIn membranous nephropathy (MN), which is characterized by deposition of immune complexes along the glomerular basement membrane (GBM), phospholipase A2 receptor (PLA2R) and thrombospondin type 1 domain-containing 7A are target antigens in approximately 70% and 1%-5% of cases of primary MN, respectively. In other cases of primary MN and in secondary MN, the target antigens are unknown.
methodsWe studied 224 cases of biopsy-proven PLA2R-negative MN and 102 controls (including 47 cases of PLA2R-associated MN) in pilot and discovery cohorts. We also evaluated 48 cases of PLA2R-negative presumed primary MN and lupus MN in a validation cohort. We used laser microdissection and mass spectrometry to identify new antigens, which were localized by immunohistochemistry.
resultsMass spectrometry detected exostosin 1 (EXT1) and exostosin 2 (EXT2) in 21 cases of PLA2R-negative MN, but not in PLA2R-associated MN and control cases. Immunohistochemistry staining revealed bright granular GBM staining for EXT1 and EXT2. Clinical and biopsy findings showed features of autoimmune disease, including lupus, in 80.7% of the 26 EXT1/EXT2-associated MN cases we identified. In the validation cohort, we confirmed that EXT1/EXT2 staining was detected in pure class 5 lupus nephritis (eight of 18 patients) and in presumed primary MN associated with signs of autoimmunity (three of 16 patients); only one of the 14 cases of mixed class 5 and 3/4 lupus nephritis was positive for EXT1/EXT2. Tests in seven patients with EXT1/EXT2-associated MN found no circulating anti-exostosin antibodies.
conclusionsA subset of MN is associated with accumulation of EXT1 and EXT2 in the GBM. Autoimmune disease is common in this group of patients.
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Registered trials
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.