Article
Pkd1 and Nek8 mutations affect cell-cell adhesion and cilia in cysts formed in kidney organ cultures.
American journal of physiology. Renal physiology - 1 Jan 2008
Natoli Thomas A, Gareski Tiffany C, Dackowski William R, Smith Laurie, Bukanov Nikolay O, Russo Ryan J, Husson Hervé, Matthews Douglas, Piepenhagen Peter, Ibraghimov-Beskrovnaya Oxana
Abstract excerpt
Development of novel therapies for polycystic kidney disease (PKD) requires assays that adequately reflect disease biology and are adaptable to high-throughput screening. Here we describe an embryonic cystic kidney organ culture model and demonstrate that a new mutant allele of the Pkd1 gene (Pkd1(tm1Bdgz)) modulates cystogenesis in this model. Cyst formation induced by cAMP is influenced by the dosage of the...
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