Patients with protein truncating PKD1 variants are at higher risk of developing ESKD early in life than patients with non-truncating PKD1 variants, and PKD2 carriers express an even milder form of ADPKD often without requirement of renal replacement therapies throughout life (Hwang et al., 2016; Bergmann et al., 2018). Here, PKD1 is linked to autosomal dominant polycystic kidney disease.