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 a gene-targeted mutation
in Pkhd1 that recapitulates phenotypic characteristics of human autosomal recessive …