Pathogenesis of Goodpasture syndrome: a molecular perspective.

Abstract:

: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.

journal_name

Semin Nephrol

journal_title

Seminars in nephrology

authors

Borza DB,Neilson EG,Hudson BG

doi

10.1053/s0270-9295(03)00131-1

subject

Has Abstract

pub_date

2003-11-01 00:00:00

pages

522-31

issue

6

eissn

0270-9295

issn

1558-4488

pii

S0270929503001311

journal_volume

23

pub_type

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