Homozygotes of PKD1 mutations caused severe phenotype with in utero onset ADPKD or premature neonate death (Cornec-Le Gall et al., 2018; Al-Hamed et al., 2019; Garel et al., 2019; Durkie et al., 2021), consistent with the embryonic lethality in homozygous Pkd1 knockout mice (Lu et al., 2001; Lantinga-van Leeuwen et al., 2007). This evidence concerns the gene PKD1 and autosomal dominant polycystic kidney disease.