Collectively, cysts originated from several nephron epithelial types, including AQP1-positive proximal tubules, ECAD-positive distal tubules, and AQP2-positive collecting ducts, in both monkeys with near-complete deletion of PKD1 and mosaic monkeys, while cyst formation in the collecting ducts was associated with cyst severity because extensive or high-density cyst formations were detected in AQP2-positive collecting ducts. Here, AQP1 is linked to cyst.