Nephrol Dial Transplant (1993) 8: 1205-1210
© 1993 European Renal Association-European Dialysis and Transplant Association
research-article
Characterization of a non-Goodpasture autoantibody to type IV collagen
1Department of Nephrology, University Hospital Lund, Sweden 2Department of Laboratory Medicine and Pathology, University of Minnesota Minnesota, Minneapolis, MN, USA 3Departrnent of Pathology, University Hospital Lund, Sweden 4Statens Serum Institut Copenhagen, Denmark
Correspondence and offprint requests to: Correspondence and offprint requests to: Charlott Johansson, Department of Nephrology, University Hospital, S-221 85 Lund, Sweden
Goodpasture's syndrome is a very severe and aggressive autoimmune kidney disease. The patients' autoantibodies, which are pathogenic, are restricted to the C-terminal region of the 
3-chain of type IV collagen. In this paper we characterize an antitype IV collagen antibody from a patient with a nonprogressive form of glomerulonephntis.
ELISA and immunoblotting were used to study the specificity of this patient's antibodies. The patient had high titres of antibodies restricted to the C-terminal region of the l-chain of type IV collagen. The antibody recognized an epitope hidden in the NC1 molecule which was fully exposed after denaturation or reduction. It was an IgG3 antibody composed of only lambda light chains, indicating that it has a potential to induce inflammatory damage and that it is probably monoclonal. This patient also had MPO-ANCA which were of IgGl subclass. Our patient had no disease progression during the 5 years of treatment.
Even though the anti-1 (IV) antibodies react with the same domain, but of a different chain of type IV collagen compared to the Goodpasture's antibodies, they do not induce any severe damage. It is thus uncertain if the anti-1 (IV) antibodies have any pathogenic role; the kidney damage might have been caused by the MPO-ANCA.
The findings support the theory that the anti-3(IV) antibody causes disease in Goodpasture's syndrome and that antibodies restricted to other subunits of the C-terminal region of type IV collagen are less harmful.
Keywords: anti-GBM nephritis; anti-type IV collagen antibodies; type IV collagen; Goodpasture's syndrome; immunoblotting; ELISA
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