Since PKHD1 was shown to localize to the primary cilium, a microtubule-based, hair-like structure that protrudes from the cell membrane and is thought to work as a critical sensory antenna [41], we set forth to investigate the ciliary phenotype of ARPKD URECs, as an additional investigation of the phenotype associated with these PKHD1 alleles. The gene discussed is PKHD1; the disease is autosomal recessive polycystic kidney disease.