Importantly, treatment with the vasopressin V2 receptor antagonist tolvaptan or the mitogen-activated extracellular signal-regulated kinase (MEK) inhibitor 2-(2-chloro-4-iodoanilino)-N-(cyclopropylmethoxy)-3,4-difluorobenzamide (CI-1040) significantly attenuated cyst growth and improved kidney morphology, confirming shared pathogenic pathways with other <i>Nphp3</i> models. Here, AVPR2 is linked to cyst.