Article
Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function.
Journal of the American Society of Nephrology : JASN - 1 Mar 2008
Kim Ingyu, Fu Yulong, Hui Kwokyin, Moeckel Gilbert, Mai Weiyi, Li Cunxi, Liang Dan, Zhao Ping, Ma Jie, Chen Xing-Zhen, George Alfred L, Coffey Robert J, Feng Zhong-Ping, Wu Guanqing
Abstract excerpt
Autosomal recessive polycystic kidney disease is caused by mutations in PKHD1, which encodes the membrane-associated receptor-like protein fibrocystin/polyductin (FPC). FPC associates with the primary cilia of epithelial cells and co-localizes with the Pkd2 gene product polycystin-2 (PC2), suggesting that these two proteins may function in a common molecular pathway. For investigation of this, a mouse model with...
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