Pathogenesis of Goodpasture syndrome: a molecular perspective.

Borza DB, Neilson EG, Hudson BG
Semin Nephrol. 2003 23 (6): 522-31

PMID: 14631560 · DOI:10.1053/s0270-9295(03)00131-1

Goodpasture (GP) syndrome is a form of anti-glomerular basement membrane (GBM) disease, in which autoantibodies bind to alpha3(IV) collagen in GBM causing rapidly progressive glomerulonephritis and pulmonary hemorrhage. The conformational GP epitopes have been mapped to 2 regions within the noncollagenous (NC1) domain of the alpha3(IV) chain. Recently, we described the molecular organization of the autoantigen in the native alpha3alpha4alpha5(IV) collagen network of the GBM. The crystal structure of the NC1 domain has revealed how the GP epitopes are sequestered in the native GBM. Further insight into the pathogenesis of disease has been obtained from better animal models. These advances provide a foundation for the development of new specific therapies.

MeSH Terms (13)

Animals Anti-Glomerular Basement Membrane Disease Antibody Specificity Autoantibodies Autoimmune Diseases Basement Membrane Disease Models, Animal Glomerulonephritis, Membranoproliferative Humans Kidney Glomerulus Mice Models, Molecular Molecular Biology

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