Article
Loss of polycystin-1 in human cyst-lining epithelia leads to ciliary dysfunction.
Journal of the American Society of Nephrology : JASN - 1 Apr 2006
Nauli Surya M, Rossetti Sandro, Kolb Robert J, Alenghat Francis J, Consugar Mark B, Harris Peter C, Ingber Donald E, Loghman-Adham Mahmoud, Zhou Jing
Abstract excerpt
A "two-hit" hypothesis predicts a second somatic hit, in addition to the germline mutation, as a prerequisite to cystogenesis and has been proposed to explain the focal nature for renal cyst formation in autosomal dominant polycystic kidney disease (ADPKD). It was reported previously that Pkd1(null/null) mouse kidney epithelial cells are unresponsive to flow stimulation. This report shows that Pkd1(+/null) cells...
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