Article
Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease.
The Journal of clinical investigation - 1 Feb 2002
Hou Xiaoying, Mrug Michal, Yoder Bradley K, Lefkowitz Elliot J, Kremmidiotis Gabriel, D'Eustachio Peter, Beier David R, Guay-Woodford Lisa M
Abstract excerpt
The congenital polycystic kidney (cpk) mutation is the most extensively characterized mouse model of polycystic kidney disease (PKD). The renal cystic disease is fully expressed in homozygotes and is strikingly similar to human autosomal recessive PKD (ARPKD), whereas genetic background modulates the penetrance of the corresponding defect in the developing biliary tree. We now describe the positional cloning,...
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