There are studies regarding the use of Tolvaptan, an approved medicine in the treatment of syndrome of inappropriate antidiuretic hormone (SIADH), and rapidly progressing autosomal dominant polycystic kidney disease (ADPKD) as a pharmacological chaperon able to inhibit the basal (constitutive) activity of V2R and it can increase the cell surface expression of the transiently expressed wild-type V2R [25]. This evidence concerns the gene AVPR2 and autosomal dominant polycystic kidney disease.