While homozygous null mice for Dzip1 (Dzip1lwpywpy/) exhibit severe ciliopathy defects, including embryonic lethality and failure to thrive postnatally, ARPKD patients with DZIP1LA90V or DZIP1LQ91H mutations only display mild PKD and hypertension, but are otherwise healthy to adulthood.[26] The drastic difference suggests that DZIP1LA90V/Q91H may specifically affect pathways implicated in PKD pathogenesis but have a benign effect on other DZIP1L‐regulated functions in cilia gating. This evidence concerns the gene DZIP1L and autosomal recessive polycystic kidney disease.