期刊
JOURNAL OF EXPERIMENTAL MEDICINE
卷 191, 期 5, 页码 899-905出版社
ROCKEFELLER UNIV PRESS
DOI: 10.1084/jem.191.5.899
关键词
Goodpasture's syndrome; type IV collagen; Fc receptor; autoimmunity; alveolar/glomerular basement membrane
The combination of hemorrhagic pneumonitis and rapidly progressive glomerulonephritis is a characteristic feature of Goodpasture's syndrome (GPS), an autoimmune disease resulting from the interaction of pathogenic anti-collagen type IV (C-IV) antibodies with alveolar and glomerular basement membranes. Lack of a suitable animal model for this fatal disease has hampered both a basic understanding of its etiology and the development of therapeutic strategies. We now report a novel model for GPS using mice deficient in a central regulatory receptor for immunoglobulin (Ig)G antibody expression and function, the type IIB Fe receptor for IgG (Fc gamma RIIB). Mutant mice immunized with bovine C-IV reproducibly develop massive pulmonary hemorrhage with neutrophil and macrophage infiltration and crescentic glomerulonephritis. The distinctive linear, ribbon-like deposition of IgG immune complex seen in GPS was observed along the glomerular and tubulointerstitial membranes of diseased animals. These results highlight the role of Fc gamma RIIB in maintaining tolerance and suggest that it may play a role in the pathogenesis of human GPS.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据