NPHP1 and ciliopathy: Increased Rho activity has been further observed in different ciliopathy conditions where ROCK inhibitors showed positive impact on cilia in vitro45, 46, 47, 48, 49 and on kidney phenotypes in mouse models.47, 48, 49 Interestingly, the ROCK inhibitor Y-27632 was shown to partially rescue ciliogenesis in NPHP1 URECs, confirming the contribution of dysregulated Rho activity signaling in NPHP1-associated phenotypes.7