{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1361699993866518656.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1681/asn.2011050464"}},{"identifier":{"@type":"URI","@value":"https://journals.lww.com/00001751-201110000-00006"}},{"identifier":{"@type":"PMID","@value":"21949093"}}],"dc:title":[{"@value":"The Pathophysiology of IgA Nephropathy"}],"description":[{"notation":[{"@value":"Here we discuss recent advances in understanding the biochemical, immunologic, and genetic pathogenesis of IgA nephropathy, the most common primary glomerulonephritis. Current data indicate that at least four processes contribute to development of IgA nephropathy. Patients with IgA nephropathy often have a genetically determined increase in circulating levels of IgA1 with galactose-deficient O-glycans in the hinge-region (Hit 1). This glycosylation aberrancy is, however, not sufficient to induce renal injury. Synthesis and binding of antibodies directed against galactose-deficient IgA1 are required for formation of immune complexes that accumulate in the glomerular mesangium (Hits 2 and 3). These immune complexes activate mesangial cells, inducing proliferation and secretion of extracellular matrix, cytokines, and chemokines, which result in renal injury (Hit 4). Recent genome-wide association studies identify five distinct susceptibility loci--in the MHC on chromosome 6p21, the complement factor H locus on chromosome 1q32, and in a cluster of genes on chromosome 22q22--that potentially influence these processes and contain candidate mediators of disease. The significant variation in prevalence of risk alleles among different populations may also explain some of the sizable geographic variation in disease prevalence. Elucidation of the pathogenesis of IgA nephropathy provides an opportunity to develop disease-specific therapies."}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1381699993866518658","@type":"Researcher","foaf:name":[{"@value":"Hitoshi Suzuki"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518662","@type":"Researcher","foaf:name":[{"@value":"Krzysztof Kiryluk"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518656","@type":"Researcher","foaf:name":[{"@value":"Jan Novak"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518663","@type":"Researcher","foaf:name":[{"@value":"Zina Moldoveanu"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518657","@type":"Researcher","foaf:name":[{"@value":"Andrew B. Herr"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518659","@type":"Researcher","foaf:name":[{"@value":"Matthew B. Renfrow"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518664","@type":"Researcher","foaf:name":[{"@value":"Robert J. Wyatt"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518660","@type":"Researcher","foaf:name":[{"@value":"Francesco Scolari"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518665","@type":"Researcher","foaf:name":[{"@value":"Jiri Mestecky"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518661","@type":"Researcher","foaf:name":[{"@value":"Ali G. Gharavi"}]},{"@id":"https://cir.nii.ac.jp/crid/1381699993866518666","@type":"Researcher","foaf:name":[{"@value":"Bruce A. Julian"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"10466673"}],"prism:publicationName":[{"@value":"Journal of the American Society of Nephrology"}],"dc:publisher":[{"@value":"Ovid Technologies (Wolters Kluwer Health)"}],"prism:publicationDate":"2011-10","prism:volume":"22","prism:number":"10","prism:startingPage":"1795","prism:endingPage":"1803"},"reviewed":"false","dcterms:accessRights":"http://purl.org/coar/access_right/c_abf2","url":[{"@id":"https://journals.lww.com/00001751-201110000-00006"}],"createdAt":"2011-09-24","modifiedAt":"2023-04-24","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=Glycosylation","dc:title":"Glycosylation"},{"@id":"https://cir.nii.ac.jp/all?q=Mesangial%20Cells","dc:title":"Mesangial Cells"},{"@id":"https://cir.nii.ac.jp/all?q=Galactose","dc:title":"Galactose"},{"@id":"https://cir.nii.ac.jp/all?q=Humans","dc:title":"Humans"},{"@id":"https://cir.nii.ac.jp/all?q=Glomerulonephritis,%20IGA","dc:title":"Glomerulonephritis, IGA"},{"@id":"https://cir.nii.ac.jp/all?q=Biomarkers","dc:title":"Biomarkers"},{"@id":"https://cir.nii.ac.jp/all?q=Immunoglobulin%20A","dc:title":"Immunoglobulin A"}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1050014128326537728","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Clinical manifestations of Henoch–Schönlein purpura nephritis and IgA nephropathy: comparative analysis of data from the Japan Renal Biopsy Registry (J-RBR)"}]},{"@id":"https://cir.nii.ac.jp/crid/1050294045368759936","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Glomerular galactose-deficient IgA1 expression analysis in pediatric patients with glomerular diseases"}]},{"@id":"https://cir.nii.ac.jp/crid/1050568772252299776","@type":"Article","resourceType":"学術雑誌論文(journal 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observational study"}]},{"@id":"https://cir.nii.ac.jp/crid/1360017282449679872","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Identification of IgA autoantibodies targeting mesangial cells redefines the pathogenesis of IgA nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360021390754353792","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"The nucleotide-sensing Toll-Like Receptor 9/Toll-Like Receptor 7 system is a potential therapeutic target for IgA nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360023720970130304","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Gross Hematuria after the COVID-19 mRNA Vaccination: Nationwide Multicenter Prospective Cohort Study in 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nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360285714472202112","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"The Xanthine Oxidase Inhibitor Febuxostat Suppresses the Progression of IgA Nephropathy, Possibly via Its Anti-Inflammatory and Anti-Fibrotic Effects in the gddY Mouse Model"}]},{"@id":"https://cir.nii.ac.jp/crid/1360294643786327680","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Aberrantly Glycosylated IgA1 in IgA Nephropathy: What We Know and What We Don’t Know"}]},{"@id":"https://cir.nii.ac.jp/crid/1360294643858018688","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"The Phenotypic Difference of IgA Nephropathy and its Race/Gender-dependent Molecular Mechanisms"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565165271598848","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Podocin is translocated to cytoplasm in puromycin aminonucleoside nephrosis rats and in poor-prognosis patients with IgA nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565166012359296","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"IgA nephropathy and IgA vasculitis with nephritis have a shared feature involving galactose-deficient IgA1-oriented pathogenesis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565166748511104","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Novel roles of complement in renal diseases and their therapeutic consequences"}]},{"@id":"https://cir.nii.ac.jp/crid/1360567182074029696","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Inhibition of STAT3 Signaling Reduces IgA1 Autoantigen Production in IgA Nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360567182452500864","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Murine Models of Human IgA Nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360567182490099584","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Association Between Post-transplantation Immunoglobulin A Deposition and Reduced Allograft Function"}]},{"@id":"https://cir.nii.ac.jp/crid/1360567184628689152","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Comprehensive 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Deposition in IgA Nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360580232420202240","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Galactose-Deficient IgA1 as a Candidate Urinary Marker of IgA Nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360580232420212992","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Special Issue: New Insights into the Pathogenesis and Therapies of IgA Nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360584340527723136","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"IgA nephropathy after COVID-19 vaccination and analysis of reported cases"}]},{"@id":"https://cir.nii.ac.jp/crid/1360584341836167680","@type":"Article","resourceType":"学術雑誌論文(journal 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IgA Nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360848657023603328","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Development of animal models of human IgA nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360848658910731136","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Cytokines Alter IgA1 O-Glycosylation by Dysregulating C1GalT1 and ST6GalNAc-II Enzymes"}]},{"@id":"https://cir.nii.ac.jp/crid/1360848658931970816","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"The role of phosphorylcholine-specific immune responses in the tonsils and peripheral blood on IgA nephropathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360848660429555328","@type":"Article","resourceType":"学術雑誌論文(journal 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