ArticleJournal of the American Society of Nephrology : JASN2008
Aberrant IgA1 glycosylation is inherited in familial and sporadic IgA nephropathy.
Article in Journal of the American Society of Nephrology : JASN, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 154 papers, 2 of them syntheses that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
154 citing papers in PubMed, 2 syntheses or guidelines pooled it, 271 citations in OpenAlex.
- Examining the association between serum galactose-deficient IgA1 and primary IgA nephropathy: a systematic review and meta-analysis.Journal of nephrology · 2024Pooled it
- Geographic differences in genetic susceptibility to IgA nephropathy: GWAS replication study and geospatial risk analysis.PLoS genetics · 2012Pooled it
- A Randomized, Controlled Trial of Rituximab in IgA Nephropathy with Proteinuria and Renal Dysfunction.Journal of the American Society of Nephrology : JASN · 2017Trial
- The combined role of galactose-deficient IgA1 and streptococcal IgA-binding M Protein in inducing IL-6 and C3 secretion from human mesangial cells: implications for IgA nephropathy.Journal of immunology (Baltimore, Md. : 1950) · 2014Trial
- The expanding role of biomarkers in the management of IgA nephropathy.Kidney international supplements · 2026Review
- IgA nephropathy: an overview of the disease, its pathophysiology, and involvement of the gut-kidney axis.Kidney international supplements · 2026Review
- IgA nephropathy new therapies: from data in adults to application in children.Pediatric nephrology (Berlin, Germany) · 2026Review
- IgA Nephropathy: Mechanisms, Risk Stratification, and Precision Therapy.Diagnostics (Basel, Switzerland) · 2026Review
- IgA nephropathy: a novel pathway in immunopathogenesis dependent on the timing of Epstein-Barr virus infection.Frontiers in immunology · 2026Review
- New Insights on IgA Nephropathy from the Perspective of Active Lesions and Systemic Inflammatory Responses.ImmunoTargets and therapy · 2026Review
- Gd-IgA1-targeting therapy in IgA nephropathy ~what is pathogenic Gd-IgA1?~.Frontiers in nephrology · 2026Review
- Autoimmunity in IgA nephropathy.Frontiers in immunology · 2026Review
- Aberrant signaling in tonsillar B cells producing pathogenicFrontiers in immunology · 2026Review
- Serum anti-β2 spectrin IgA antibodies are produced in a CD4 T cell-independent manner in IgA nephropathy model mouse.Frontiers in immunology · 2026Article
- Review
- Serum IgA isotypes are associated with percent emphysema, wall thickness and lung function decline.Thorax · 2025Article
- Glomerular Galactose-Deficient IgA1 Deposition in 192 Autopsy Cases in Japan.Nephrology (Carlton, Vic.) · 2025Article
- Predictive value of Gd-IgA1, poly-IgA in the treatment of IgA nephropathy with targeted-release formulation budesonide.Clinical kidney journal · 2025Article
- Expected and verified benefits from old and new corticosteroid treatments in IgA nephropathy: from trials in adults to new IPNA-KDIGO guidelines.Pediatric nephrology (Berlin, Germany) · 2025Review
- IgA vasculitis with nephritis accompanied by pulmonary tuberculosis: a case report.CEN case reports · 2025Article
94 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 1 country.
Funding
Abstract
IgA nephropathy (IgAN) is a complex trait determined by genetic and environmental factors. Most IgAN patients exhibit a characteristic undergalactosylation of the O-glycans of the IgA1 hinge region, which promotes formation and glomerular deposition of immune complexes. It is not known whether this aberrant glycosylation is the result of an acquired or inherited defect, or whether the presence of aberrant IgA1 glycoforms alone can produce IgAN. A newly validated lectin enzyme-linked immunosorbent assay (ELISA) was used to determine the serum level of galactose-deficient IgA1 (Gd-IgA1) in a cohort of 89 IgAN patients and 266 of their relatives. High Gd-IgA1 levels (> or =95th percentile for controls) were observed in all 5 available patients with familial IgAN, in 21 of 45 (47%) of their at-risk relatives (assuming autosomal dominant inheritance), and in only 1 of 19 (5%) of unrelated individuals who married into the family. This provides evidence that abnormal IgA1 glycosylation is an inherited rather than acquired trait. Similarly, Gd-IgA1 levels were high in 65 of 84 (78%) patients with sporadic IgAN and in 50 of 202 (25%) blood relatives. Heritability of Gd-IgA1 was estimated at 0.54 (P = 0.0001), and segregation analysis suggested the presence of a major dominant gene on a polygenic background. Because most relatives with abnormal IgA1 glycoforms were asymptomatic, additional cofactors must be required for IgAN to develop. The fact that abnormal IgA1 glycosylation clusters in most but not all families suggests that measuring Gd-IgA1 may help distinguish patients with different pathogenic mechanisms of disease.
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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.