Dysregulation of the V2R-generated cAMP pool in ADPKD is believed to influence fluid secretion and cell proliferation, which promotes cystogenesis (Cassola et al., 1993; Meijer et al., 2011; Reif et al., 2011; Rinschen et al., 2014). Here, AVPR2 is linked to autosomal dominant polycystic kidney disease.