Analysis of livers from ADPKD patients and autosomal-dominant and autosomal-recessive Cpk-mutant mouse models of polycystic kidney disease, which develop cysts as a result of mutations in cystin-1, revealed that, of the various NOTCH pathway members, NOTCH3 was consistently upregulated in cyst-lining epithelial cells (Idowu et al., 2018). This evidence concerns the gene NOTCH3 and autosomal dominant polycystic kidney disease.