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

H Holthöfer, H Ahola, ML Solin, S Wang… - The American journal of …, 1999 - Elsevier
H Holthöfer, H Ahola, ML Solin, S Wang, T Palmen, P Luimula, A Miettinen, D Kerjaschki
The American journal of pathology, 1999Elsevier
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 …
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.
Elsevier