Pathogenesis of Goodpasture Syndrome: A Molecular Perspective

Dorin Bogdan Borza*, Eric G. Neilson, Billy G. Hudson

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

48 Scopus citations

Abstract

Goodpasture (GP) syndrome is a form of anti-glomerular basement membrane (GBM) disease, in which autoantibodies bind to α3(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 α3(IV) chain. Recently, we described the molecular organization of the autoantigen in the native α3α 4α5(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.

Original languageEnglish (US)
Pages (from-to)522-531
Number of pages10
JournalSeminars in nephrology
Volume23
Issue number6
DOIs
StatePublished - Nov 2003

ASJC Scopus subject areas

  • Nephrology

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