{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T08:59:05Z","timestamp":1790845145734,"version":"4.1.0"},"reference-count":115,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Nephrol."],"abstract":"<jats:p>IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis worldwide, and galactose-deficient IgA1 (Gd-IgA1) is widely recognized as the central pathogenic molecule in the current multi-hit model of IgAN. Over the past few decades, advances in our understanding of IgAN pathogenesis have driven the development of mechanism-based therapies targeting Gd-IgA1-producing cells and the pathways that regulate their differentiation and survival. Recent clinical trials have demonstrated marked reductions in circulating Gd-IgA1, accompanied by significant improvements in proteinuria and preservation of kidney function, highlighting the transition from non-specific disease-modifying therapies toward mechanism-based therapies in IgAN. However, optimizing the use of these emerging therapies requires a deeper understanding of the immune mechanisms responsible for pathogenic Gd-IgA1 production. In this review, we summarize current knowledge regarding the cellular sources and molecular pathways responsible for the production of Gd-IgA1, review recent advances in therapies targeting these cells, and discuss the emerging concept that specific Gd-IgA1 fractions, including IgA-type anti-mesangial cell autoantibodies (IgA-MESCA), may contribute to the disease pathogenesis.<\/jats:p>","DOI":"10.3389\/fneph.2026.1977707","type":"journal-article","created":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T08:26:49Z","timestamp":1790843209000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Gd-IgA1-targeting therapy in IgA nephropathy ~what is pathogenic Gd-IgA1?~"],"prefix":"10.3389","volume":"6","author":[{"given":"Kazuaki","family":"Mori","sequence":"first","affiliation":[{"name":"Department of Nephrology, Juntendo University Faculty of Medicine","place":["Tokyo, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihito","family":"Nihei","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Juntendo University Faculty of Medicine","place":["Tokyo, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Juntendo University Faculty of Medicine","place":["Tokyo, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,10,1]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.2215\/cjn.07420716","article-title":"IgA nephropathy","volume":"12","author":"Rodrigues","year":"2017","journal-title":"Clin J Am Soc Nephrol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1007\/s10157-021-02095-8","article-title":"A digest from evidence-based clinical practice guideline for IgA nephropathy 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