Since Nphp1‐ and Nphp4‐mutant mice display only eye and male reproductive organ defects (Jiang et al, 2008, 2009; Louie et al, 2010; Won et al, 2011) and Invs‐mutant mouse embryos exhibit kidney cysts and left–right asymmetry defects but, for example, no brain defects (Mochizuki et al, 1998; Morgan et al, 1998), we deduce that the very severe ciliopathy phenotype of Rpgrip1l−/− mouse embryos is not or not completely caused by the impact of Rpgrip1l deficiency on the Nphp4‐Invs‐Nphp1 axis. Here, NPHP1 is linked to Kidney Cyst.