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Human ADPKD primary cyst epithelial cells with a novel PKD1 mutation exhibit defective ciliary polycystin localization and loss of flow-induced Ca(2+) signaling

ADPKD gene products polycystin-1 (PC1) and polycystin-2 (PC2) colocalize in the apical monocilia of renal epithelial cells. Mouse and human renal cells without PC1 protein show impaired ciliary mechanosensation, and this impairment has been proposed to promote cystogenesis. However, most cyst epithe...

Ausführliche Beschreibung

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Bibliographische Detailangaben
Hauptverfasser: Xu, Chang, Rossetti, Sandro, Jiang, Lianwei, Harris, Peter C., Brown-Glaberman, Ursa, Wandinger-Ness, Angela, Bacallao, Robert, Alper, Seth L.
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2006
Schlagworte:
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3586432/
https://ncbi.nlm.nih.gov/pubmed/17090781
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajprenal.00285.2006
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