After Pkd1 knockout, mice were orally administered KM-CP-met, KM-met, free metformin, or KM-CP every three days at a metformin concentration of 300 mg/kg/day (Fig. 5A), as reported previously in ADPKD preclinical studies (15, 17, 36), and euthanized on P120 when severe disease is expected (57). Here, PKD1 is linked to autosomal dominant polycystic kidney disease.