Journal Article

Identification of post-transplant anti-α5(IV) collagen alloantibodies in X-linked Alport syndrome

P. Dehan, L. P. W. J. Van Den Heuvel, H. J. M. Smeets, K. Tryggvason and J.-M. Foidart

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 11, issue 10, pages 1983-1988
Published in print October 1996 | ISSN: 0931-0509
e-ISSN: 1460-2385 | DOI:
Identification of post-transplant anti-α5(IV) collagen alloantibodies in X-linked Alport syndrome

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X-linked Alport syndrome (AS) is a heritable disorder which is associated with mutations in the type IV collagen α5(IV) chain gene (COL4AS) located on chromosome X. Following renal transplantation, an average of 6% of male AS patients develop anti-GBM nephritis. We studied the specificity of the antibodies against type IV collagen in the serum of a patient with COL4A5 partial deletion. The specificity of these alloantibodies was determined against collagenasedigested GBM, as well as against recombinant noncollagenous (NCl) domains of the type IV collagen α1(IV)—α6(IV) chains expressed in Escherichia coli. Immunoblotting and ELISA demonstrated that these antibodies bound specifically to the NCl domain of α5(IV) collagen. There was no binding to the NCl domain of the other chains, including the Goodpasture antigen. Competitive ELISA confirmed the results obtained by ELISA and immunoblotting. This patient developed alloantibodies directed against antigens present in the grafted kidney, but absent from his Alport kidney. The pathogenesis of post-transplantation glomerulonephritis in the Alport patient studied is thus similar to that of Goodpasture syndrome, with the exception that the pathogenic antibodies are targeted to another α chain of type IV collagen.

Keywords: Alport syndrome; anti-GBM nephritis; type IV collagen; Goodpasture antigen; kidney transplantation

Journal Article.  0 words. 

Subjects: Nephrology

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