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      Recurrence of Nephrotic Syndrome after Transplantation in CNF Is due to Autoantibodies to Nephrin

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          The novel gene NPHS1 is defective in the patients with congenital nephrotic syndrome of the Finnish type (CNF) leading to abnormal expression of the respective protein product nephrin in glomerular cells. CNF patients are treated with early nephrectomy and renal transplantation, but about 20% show recurrence of nephrotic syndrome (NS). We used indirect immunofluorescence microscopy and immunoblotting and an ELISA assay to search for circulating autoantibodies to nephrin, the protein defect in CNF patient kidneys. In serial serum samples gathered before and after recurrence of NS, we show an increased antibody titer to nephrin prior to the NS episode and a subsequent drop in antibody level after its successful treatment and reactivity of the high titer sera with glomeruli in indirect immunofluorescence microscopy as well. The results show that the transplantation treatment introduces a neoantigen inducing production of autoantibodies, which may be pathogenic for perturbation of the function of the glomerular filtration barrier.

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          Structure of the Gene for Congenital Nephrotic Syndrome of the Finnish Type (NPHS1) and Characterization of Mutations

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            • Abstract: not found
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            Molecular Cloning of pTAC12 an Alternative Splicing Product of the CD3γ Chain as a Component of the Pre-T Cell Antigen-Receptor Complex


              Author and article information

              Nephron Exp Nephrol
              Cardiorenal Medicine
              S. Karger AG
              31 August 2001
              : 9
              : 5
              : 327-331
              Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki, Finland
              52628 Exp Nephrol 2001;9:327–331
              © 2001 S. Karger AG, Basel

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              Figures: 4, Tables: 1, References: 20, Pages: 5
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