ArticleJournal of the American Society of Nephrology : JASN2018
DnaJ Homolog Subfamily B Member 9 Is a Putative Autoantigen in Fibrillary GN.
Article in Journal of the American Society of Nephrology : JASN, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 56 papers.
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Who cites it
56 citing papers in PubMed, 98 citations in OpenAlex.
- DNAJB9 in Fibrillary Glomerulonephritis: Diagnostic Biomarker, Putative Autoantigen, or Disease-Associated Scaffold? A Case-Based Narrative Review.Life (Basel, Switzerland) · 2026Article
- Clinicopathologic features and outcomes of patients with DNAJB9-positive fibrillary glomerulonephritis.Clinical kidney journal · 2026Article
- Article
- DNAJB9 Fibrillary Glomerulonephritis Following Rituximab-Based Therapy: A Case of Temporary Renal Recovery.Cureus · 2025Article
- Fibrillary glomerulonephritis: an observational study of clinical-pathological features and outcomes in patients from a multi-institutional cohort.Clinical kidney journal · 2025Article
- Therapeutic Challenges and New Era in Fibrillary Glomerulonephritis with the Introduction of DNAJB9: Experience from a Tertiary Nephrology Center.Journal of clinical medicine · 2025Article
- Fibrillary Glomerulonephritis Diagnosis Is Enhanced by DNAJB9: Three Cases with Different Clinical, Anatomopathologic Features and Outcomes.Pathophysiology : the official journal of the International Society for Pathophysiology · 2025Article
- Fibrillary glomerulonephritis: A retrospective analysis of a case series from a tertiary center.Clinical nephrology · 2025Article
- Review
- Non-Invasive Biomarkers for Diagnosis, Risk Prediction, and Therapy Guidance of Glomerular Kidney Diseases: A Comprehensive Review.International journal of molecular sciences · 2024Review
- A proteomic atlas of kidney amyloidosis provides insights into disease pathogenesis.Kidney international · 2024Article
- IgA-dominant glomerulonephritis with DNAJB9-negative fibrillar polytypic immunoglobulin deposits in the subepithelium.CEN case reports · 2023Article
- DNAJB9-Positive Tubulointerstitial-Predominant Fibrillary Nephritis.Kidney international reports · 2023Article
- DNAJB9 Is a Reliable Immunohistochemical Marker of Fibrillary Glomerulonephritis: Evaluation of Diagnostic Efficacy in a Large Series of Kidney Biopsies.Biomedicines · 2022Article
- Tandem Mass Spectrometry-Based Amyloid Typing Using Manual Microdissection and Open-Source Data Processing.American journal of clinical pathology · 2022Article
- Filgrastim-induced recurrent macroscopic hematuria in a patient with DNAJB9 positive fibrillary glomerulonephritis.Journal of nephrology · 2022Article
- The Proteome of Antibody-Mediated Rejection: From Glomerulitis to Transplant Glomerulopathy.Biomedicines · 2022Article
- Fibrillary Glomerulonephritis and Monoclonal Gammopathy: Potential Diagnostic Challenges.Frontiers in oncology · 2022Review
- Fibrillary Glomerulonephritis, DNAJB9, and the Unfolded Protein Response.Glomerular diseases · 2022Review
- The function of the co-chaperone ERdj4 in diverse (patho-)physiological conditions.Cellular and molecular life sciences : CMLS · 2021Review
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Abstract
Fibrillary GN is a rare form of GN of uncertain pathogenesis that is characterized by the glomerular accumulation of randomly arranged, nonbranching fibrils (12-24 nm) composed of Ig and complement proteins. In this study, we used mass spectrometry to comprehensively define the glomerular proteome in fibrillary GN compared with that in controls and nonfibrillary GN renal diseases. We isolated glomeruli from formalin-fixed and paraffin-embedded biopsy specimens using laser capture microdissection and analyzed them with liquid chromatography and data-dependent tandem mass spectrometry. These studies identified DnaJ homolog subfamily B member 9 (DNAJB9) as a highly sampled protein detected only in fibrillary GN cases. The glomerular proteome of fibrillary GN cases also contained IgG1 as the dominant Ig and proteins of the classic complement pathway. In fibrillary GN specimens only, immunofluorescence and immunohistochemistry with an anti-DNAJB9 antibody showed strong and specific staining of the glomerular tufts in a distribution that mimicked that of the immune deposits. Our results identify DNAJB9 as a putative autoantigen in fibrillary GN and suggest IgG1 and classic complement effector pathways as likely mediators of the destructive glomerular injury in this disease.
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