Journal
SEMINARS IN IMMUNOPATHOLOGY
Volume 43, Issue 5, Pages 669-678Publisher
SPRINGER HEIDELBERG
DOI: 10.1007/s00281-021-00883-8
Keywords
Aberrantly glycosylated IgA1; IgG autoantibodies; Immune complex; Mucosal immunity; APRIL
Categories
Funding
- NIH [DK078244, AI149431, DK082753, GM098539]
- JSPS KAKENHI [21K08285]
- Grants-in-Aid for Scientific Research [21K08285] Funding Source: KAKEN
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IgA nephropathy is the most common primary glomerulonephritis worldwide, with its pathogenesis mainly related to aberrant glycoforms of IgA1 and the formation of immune complexes. Complement activation likely plays a crucial role in the pathogenic properties of these complexes.
IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. This disease, discovered in 1968, is characterized by IgA-IgG glomerular immunodeposits with a mesangial pattern. It is thought that these immunodeposits originate from the immune complexes formed in the circulation. It is hypothesized that the pathogenesis of IgAN is driven by aberrant glycoforms of IgA1 (galactose-deficient IgA1, Gd-IgA1). Gd-IgA1, in genetically susceptible individuals, represents the initiating factor for the formation of circulating immune complexes due to its recognition by IgG autoantibodies and the subsequent formation of pathogenic IgA1-IgG immune complexes. Complement activation through alternative and/or lectin pathways is likely playing an important role in the pathogenic properties of these complexes and may further upregulate local inflammatory responses and glomerular injury.
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