CLCN5 and Dent disease: Inactivating mutations of CLCN5 in Dent disease patients (15) as well as the deletion of CLCN5 in knock-out (KO) mice (16,17) lead to severe LMWP due to a defective endocytic uptake in PTCs, which has been associated with the disappearance of megalin and cubilin at the brush border of PTCs.