The renal phenotype of Pkhd1‐mutant mice differs from the presentation of ARPKD in humans, varying from a complete absence of kidney disease to slowly progressing late‐onset renal cysts (Ishimoto et al., 2023; Williams et al., 2008; Woollard et al., 2007; Yang et al., 2023), thus Cpk mice are widely used to model ARPKD (Chiu et al., 2006; Huang et al., 2016). This evidence concerns the gene PIK3C2A and cystic kidney disease.