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(American Journal of Pathology. 1999;155:1681-1687.)
© 1999 American Society for Investigative Pathology

Regular Articles

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* and Dontscho Kerjaschki{dagger}

From the Division of Bacteriology and Immunology,*
Haartman Institute, University of Helsinki, Helsinki, Finland; and the Division of Ultrastructural Pathology and Cell Biology,{dagger}
Institute of Clinical Pathology, University of Vienna, Vienna, Austria

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.




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