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Published online 6 March 2000.
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© The Rockefeller University Press, 0022-1007/2000/3/899/ $5.00
The Journal of Experimental Medicine, Volume 191, Number 5, March 6, 2000 899-906

Brief Definitive Report

Fc{gamma} Receptor IIB–deficient Mice Develop Goodpasture's Syndrome upon Immunization with Type IV Collagen: A Novel Murine Model for Autoimmune Glomerular Basement Membrane Disease

Akira Nakamuraa,b,c, Takae Yuasaa,c, Azusa Ujikea,c, Masao Onoa,c, Toshihiro Nukiwab, Jeffrey V. Ravetchd, and Toshiyuki Takaia,c
a Department of Experimental Immunology, Institute of Development, Aging and Cancer, Tohoku University, Sendai 980-8575, Japan
b Department of Respiratory Oncology and Molecular Medicine, Institute of Development, Aging and Cancer, Tohoku University, Sendai 980-8575, Japan
c Core Research for Evolutional Science and Technology (CREST), Japan Science and Technology Corporation (JST), Tokyo 101-0062, Japan
d Laboratory of Molecular Genetics and Immunology, The Rockefeller University, New York, New York 10021

Correspondence to: Toshiyuki Takai, Dept. of Experimental Immunology, Institute of Development, Aging and Cancer, Tohoku University, 4-1 Seiryo, Sendai 980-8575, Japan. Tel:81-22-717-8501 Fax:81-22-717-8505 E-mail:tostakai{at}idac.tohoku.ac.jp.

Released online: 6 March 2000

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 Fc 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.

Key Words: Goodpasture's syndrome, type IV collagen, Fc receptor, autoimmunity, alveolar/glomerular basement membrane


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