Molecular Architecture of the Goodpasture Autoantigen in Anti-GBM Nephritis (original) (raw)

Goodpasture's disease is characterized by autoantibodies binding to the NC1 domain of type IV collagen within the glomerular basement membrane, leading to severe kidney and lung pathology. This study compares autoantibody epithelial binding in Goodpasture's disease to alloantibody binding in Alport's post-transplantation nephritis. Findings reveal differing antibody conformations; while autoantibodies in Goodpasture's require dissociation of the hexamer for binding, alloantibodies in Alport's bind to native epitopes, suggesting distinct pathogenic mechanisms and implicating high antibody titers in renal dysfunction.