Article
Cystogenesis in ARPKD results from increased apoptosis in collecting duct epithelial cells of Pkhd1 mutant kidneys.
Experimental cell research - 15 Jan 2011
Hu Bo, He Xiusheng, Li Ao, Qiu Qingchao, Li Cunxi, Liang Dan, Zhao Ping, Ma Jie, Coffey Robert J, Zhan Qimin, Wu Guanqing
Abstract excerpt
Mutations in the PKHD1 gene result in autosomal recessive polycystic kidney disease (ARPKD) in humans. To determine the molecular mechanism of the cystogenesis in ARPKD, we recently generated a mouse model for ARPKD that carries a targeted mutation in the mouse orthologue of human PKHD1. The homo...
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