The genetics and immunobiology of IgA nephropathy.

Jan Novak, Krzysztof Kiryluk

Journal: The Journal of clinical investigation 2014;124(6):2325-32

PMID: 24892706

Free Full Text on American Society for Clinical Investigation View on PubMed

Abstract

IgA nephropathy (IgAN) represents the leading cause of kidney failure among East Asian populations and the most frequent form of primary glomerulonephritis among Europeans. Patients with IgAN develop characteristic IgA1-containing immune complexes that deposit in the glomerular mesangium, producing progressive kidney injury. Recent studies define IgAN as an autoimmune trait of complex architecture with a strong genetic determination. This Review summarizes new insights into the role of the O-glycosylation pathway, anti-glycan immune response, mucosal immunity, antigen processing and presentation, and the alternative complement pathway in the pathogenesis of IgAN.

DOI: 10.1172/JCI74475

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MeSH Terms: Amino Acid Sequence, Antigen Presentation, Antigen-Antibody Complex, Complement Pathway, Alternative, Genome-Wide Association Study, Glomerulonephritis, IGA, Glycosylation, Humans, Immunity, Mucosal, Immunoglobulin A, Models, Genetic, Models, Immunological, Molecular Sequence Data, Molecular Structure