期刊
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
卷 24, 期 2, 页码 253-267出版社
AMER SOC NEPHROLOGY
DOI: 10.1681/ASN.2012060582
关键词
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资金
- Else Kroner-Fresenius-Stiftung
- Fritz-Thyssen-Stiftung
- Deutsche Forschungsgemeinschaft
- Excellence Initiative of the German Federal Government [EXC 294]
- Excellence Initiative of the German State Government [EXC 294]
- Deutsche Nierenstiftung
Precise positioning of the highly complex interdigitating podocyte foot processes is critical to form the normal glomerular filtration barrier, but the molecular programs driving this process are unknown. The protein atypical protein kinase C (aPKC) a component of the Par complex, which localizes to tight junctions and interacts with slit diaphragm proteins may play a role. Here, we found that the combined deletion of the aPKC lambda/iota and aPKC zeta isoforms in podocytes associated with incorrectly positioned centrosomes and Golgi apparatus and mislocalized molecules of the slit diaphragm. Furthermore, aPKC-deficient podocytes failed to form the normal network of foot processes, leading to defective glomerular maturation with incomplete capillary formation and mesangiolysis. Our results suggest that aPKC isoforms orchestrate the formation of the podocyte processes essential for normal glomerular development and kidney function. Defective aPKC signaling results in a dramatically simplified glomerular architecture, causing severe proteinuria and perinatal death. J Am Soc Nephrol 24: 253-267, 2013. doi: 10.1681/ASN.2012060582
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