AVPR2 and autosomal recessive polycystic kidney disease: Based on the strong AVP deregulation observed in ADPKD and ARPKD animal models, strategic reduction of AVP-mediated signaling and intracellular cAMP using V2R antagonists was found to reduce both cystic fluid secretion and cyst size in murine PKD models [122,342,343] and PKD patients [130,136,321].