Genetic defects in TFAP2B/Tfap2b genes result in ARPKD-like phenotype in mice (Moser et al., 1997a; Moser et al., 2003; Wang et al., 2018; Lamontagne et al., 2022) while humans with Char syndrome have no renal phenotype.6 The gene discussed is TFAP2B; the disease is autosomal recessive polycystic kidney disease.