Renal cysts and fibrosis caused by epithelial cell polarity defects in mice lacking mammalian Lin‐7c (MALS‐3)

Polarity is a physical attribute of most eukaryotic cells that is indispensable for their function. In mammalian kidney, polarization of epithelial cells that line renal tubules allows for the vectorial transport of solutes that result in urine formation. Genetic studies show that polarization of in...

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Bibliographic Details
Published in:The FASEB journal 2007-04, Vol.21 (5), p.A544-A544
Main Authors: Olsen, Olav, Funke, Lars, Long, Jia‐fu, Fukata, Masaki, Nicoll, Roger A, Zhang, Mingjie, Bredt, David S
Format: Article
Language:English
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Summary:Polarity is a physical attribute of most eukaryotic cells that is indispensable for their function. In mammalian kidney, polarization of epithelial cells that line renal tubules allows for the vectorial transport of solutes that result in urine formation. Genetic studies show that polarization of invertebrate epithelia requires the crumbs, PAR‐3 and discs large complexes, which comprise primarily PDZ domain proteins. These evolutionarily conserved protein complexes occur in mammalian kidney; however, their role in renal development remains poorly defined. Here, we find the small PDZ protein mammalian LIN‐7c (MALS‐3) promotes apico‐basal polarization of the crumbs and DLG complexes. An inability to properly polarize these complexes in MALS‐3 knockout mice results in renal hypoplasia, fibrosis and cysts.
ISSN:0892-6638
1530-6860
DOI:10.1096/fasebj.21.5.A544-b