PKD1 and cystic kidney disease: In conclusion, PKD1insG/+ pigs showing 100% penetrance of a cystic kidney phenotype have many symptomatic similarities to patients with ADPKD caused by heterozygous mutation of PKD1. Similarities include the formation of macroscopic renal cysts during the neonatal stage, number and cystogenic dynamics of renal cysts formed, pathological features such as interstitial fibrosis, and presence of an asymptomatic prematuration stage.