Article
Polycystin-2 expression is regulated by a PC2-binding domain in the intracellular portion of fibrocystin.
The Journal of biological chemistry - 14 Nov 2008
Kim Ingyu, Li Cunxi, Liang Dan, Chen Xing-Zhen, Coffy Robert J, Ma Jie, Zhao Ping, Wu Guanqing
Abstract excerpt
Autosomal dominant (ADPKD) and autosomal recessive (ARPKD) polycystic kidney disease are caused by mutations in Pkd1/Pkd2 and Pkhd1, which encode polycystins (PCs) and fibrocystin/polyductin (FPC). Our recent study reported that a deficiency in FPC increases the severity of cystic disease in Pkd2 mutants and down-regulates PC2 in vivo, but the precise molecular mechanism of these effects is unknown (Kim, I., Fu,...
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