They include ClC-5 knock-out (KO) [9,10,11,12], knock-in (KI) mice harboring the artificial E211A mutation (inducing a Dent disease phenotype but never described in patients) [13] and the pathogenic N340K mutation (described in patients) [14] (Table 1), knock-down of the ClC-5 homolog in Drosophila melanogaster [15], cells carrying ClC-5 mutants derived from DD type 1 patients (ciPTECs) [16,17], Xenopus laevis oocytes, and different stably or transiently transfected cell lines [12,18,19,20,21,22,23,24,25]. This evidence concerns the gene CLCN5 and Dent disease.