While we used mice spanning the full spectrum of ADPKD severity seen in patients, from early-onset (C57BL/6J Pkd1RC/–) to late-onset (C57BL/6J Pkd1RC/RC) PKD, the underlying disease driver was always a germline modification impacting polycystin-1 dosage. Here, PKD1 is linked to autosomal dominant polycystic kidney disease.