Defective oriented cell division was also identified in the pck rat model of ARPKD (Fischer et al., 2006), which develops cystic renal disease by 3 weeks of age due to decreased expression of Pkhd1. Complementing these findings, kidney lysates from paediatric patients with ARPKD and ESKD contain an increased expression of PCP protein-encoding transcripts, including WNT5A, VANGL2, ATMIN, and SCRIB, compared to age-matched controls (Figure 3; Richards et al., 2019). This evidence concerns the gene PKHD1 and autosomal recessive polycystic kidney disease.