Recurrence of nephrotic syndrome after transplantation in CNF is due to autoantibodies to nephrin

Shi Xuan Wang, Heikki Ahola, Tuula Palmen, Marja Liisa Solin, Pauliina Luimula, Harry Holthöfer

Research output: Contribution to journalArticlepeer-review

38 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)327-331
Number of pages5
JournalExperimental Nephrology
Issue number5
StatePublished - 2001
Externally publishedYes


  • Congenital nephrotic syndrome
  • Finnish type
  • Nephrin
  • Nephrotic syndrome, recurrence

ASJC Scopus subject areas

  • Nephrology


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