CareLit Fachartikel

The polarity protein scribble interacts with Neph1 during glomerular development

M Schmidts, B Hartleben, B Mayer, L Schneider, N Wanner, E Widmeier, G Walz, T Huber · Klinische Pädiatrie · 2011 · Heft 03

Dokument
866410
CareLit-ID
Jahr
2011
Publikation
PDF
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Metadaten
DOI
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Bibliografische Angaben

Zeitschrift
Klinische Pädiatrie
Autor:innen
M Schmidts, B Hartleben, B Mayer, L Schneider, N Wanner, E Widmeier, G Walz, T Huber
Ausgabe
Heft 03 / 2011
Seiten
–
Erschienen: 2011-03-23 00:00:00
ISSN
0300-8630
DOI
10.1055/s-0031-1273781

Zusammenfassung

Objectives and Study: The glomerular filtration barrier is a unique structure characterized by a specialized three-dimensional framework of podocytes. Mutations in genes encoding for slitdiaphragm proteins Nephrin (NPHS1) and Podocin (NPHS2) cause a hereditary nephrotic syndrome in humans and genetic deletion of Neph1, a slit diaphragm protein closely related toNephrin, leads to congenital nephrotic syndrome in mice. An apico-basal polarity axis allows for podocyte orientation between the urinary space and the glomerular basement membrane. Our recent studies document that conserved polarity protein complexes suc…

Schlagworte

Neph1 Scribble glomerular development nephrotic syndrome podocyte Klinische Pädiatrie