Nephrin localizes at the podocyte filtration slit area and is characteristically spliced in the human kidney

Harry Holthöfer, Heikki Ahola, Marja Liisa Solin, Shixuan Wang, Tuula Palmen, Pauliina Luimula, Aaro Miettinen, Dontscho Kerjaschki

Research output: Contribution to journalArticle

156 Scopus citations

Abstract

Defects in the newly reported gene NPHS1 in chromosome 19 cause the massive proteinuria of Finnish type congenital nephrotic syndrome (CNF). Together with its gene product, nephrin, NPHS1 is providing new understanding of the pathophysiological mechanisms of glomerular filtration. Here we show the characteristic splicing of NPHS1 mRNA in the normal and CNF kidneys and localize nephrin exclusively in the glomerulus and to the filtration slit area by light and immunoelectron microscopy. These results indicate that nephrin is a new protein of the interpodocyte filtration slit area with a profound role in the pathophysiology of the filtration barrier.

Original languageEnglish (US)
Pages (from-to)1681-1687
Number of pages7
JournalAmerican Journal of Pathology
Volume155
Issue number5
DOIs
StatePublished - Nov 1999

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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    Holthöfer, H., Ahola, H., Solin, M. L., Wang, S., Palmen, T., Luimula, P., Miettinen, A., & Kerjaschki, D. (1999). Nephrin localizes at the podocyte filtration slit area and is characteristically spliced in the human kidney. American Journal of Pathology, 155(5), 1681-1687. https://doi.org/10.1016/S0002-9440(10)65483-1