Molecular Analysis of Goodpasture's Disease Following Hematopoietic Stem Cell Transplant in a Pediatric Patient, Recalls the Conformeropathy of Wild-Type Anti-GBM Disease

• Published in: Frontiers in immunology Vol. 10; p. 2659
• Main Authors: Gray, Paul E, McCarthy, Hugh, Siggs, Owen M, Saleem, Moin A, O' Brien, Tracy, Frith, Katie, Ziegler, John B, Kitching, A Richard, Fogo, Agnes B, Hudson, Billy G, Pedchenko, Vadim
• Format: Journal Article
• Language: English
• Published: Switzerland Frontiers Media S.A 14.11.2019
• Subjects: Allografts; alloimmunity; anti-GBM; Anti-Glomerular Basement Membrane Disease - etiology; Anti-Glomerular Basement Membrane Disease - immunology; Autoantibodies - immunology; Autoantigens - immunology; Child; Collagen Type IV - genetics; Collagen Type IV - immunology; conformeropathy; Epitopes - immunology; glomerular basement membrane; Goodpastures; GvHD; Hematopoietic Stem Cell Transplantation - adverse effects; Humans; Immunology; Isoantibodies - immunology; Lymphoproliferative Disorders - therapy; Male; Postoperative Complications - etiology; Postoperative Complications - immunology; alloimmunity; conformeropathy; Goodpastures; autoimmunity; GvHD; anti-GBM; glomerular basement membrane

Goodpasture's disease (GP) is mediated by autoantibodies that bind the glomerular and alveolar basement membrane, causing rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. The autoantibodies bind neoepitopes formed upon disruption of the quaternary structure of α345NC...