Article
Human polycystin-2 transgene dose-dependently rescues ADPKD phenotypes in Pkd2 mutant mice.
The American journal of pathology - 1 Oct 2015
Li Ao, Tian Xin, Zhang Xiaoli, Huang Shunwei, Ma Yujie, Wu Dianqing, Moeckel Gilbert, Somlo Stefan, Wu Guanqing
Abstract excerpt
Although much is known about the molecular genetic mechanisms of autosomal-dominant polycystic kidney disease (ADPKD), few effective treatment is currently available. Here, we explore the in vivo effects of causal gene replacement in orthologous gene models of ADPKD in mice. Wild-type mice with human PKD2 transgene (PKD2(tg)) overexpressed polycystin (PC)-2 in several tissues, including the kidney and liver, and...
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