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

doi:10.1016/S0002-9440(10)65483-1

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 journal › Article › peer-review

165 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 language	English (US)
Pages (from-to)	1681-1687
Number of pages	7
Journal	American Journal of Pathology
Volume	155
Issue number	5
DOIs	https://doi.org/10.1016/S0002-9440(10)65483-1
State	Published - Nov 1999
Externally published	Yes

ASJC Scopus subject areas

Pathology and Forensic Medicine

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10.1016/S0002-9440(10)65483-1

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title = "Nephrin localizes at the podocyte filtration slit area and is characteristically spliced in the human kidney",

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.",

author = "Harry Holth{\"o}fer and Heikki Ahola and Solin, {Marja Liisa} and Shixuan Wang and Tuula Palmen and Pauliina Luimula and Aaro Miettinen and Dontscho Kerjaschki",

note = "Funding Information: Supported by the Academy of Finland, a research grant from Helsinki University Hospital, the Finnish Foundation of Heart Disease, and the Sigrid Juselius Foundation. ",

year = "1999",

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T1 - Nephrin localizes at the podocyte filtration slit area and is characteristically spliced in the human kidney

AU - Holthöfer, Harry

AU - Ahola, Heikki

AU - Solin, Marja Liisa

AU - Wang, Shixuan

AU - Palmen, Tuula

AU - Luimula, Pauliina

AU - Miettinen, Aaro

AU - Kerjaschki, Dontscho

N1 - Funding Information: Supported by the Academy of Finland, a research grant from Helsinki University Hospital, the Finnish Foundation of Heart Disease, and the Sigrid Juselius Foundation.

PY - 1999/11

Y1 - 1999/11

N2 - 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.

AB - 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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JO - American Journal of Pathology

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IS - 5

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

Abstract

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