Heterozygotes exhibit distal-tubules-derived small cysts. a Low-power, H&E-stained images and ultrasonography image of heterozygous kidneys. Scale bars in large H&E-stained images, 1 mm. Scale bar in the small box, 100 µm. Scale bar in the ultrasonography image, 10 mm. b Expressions of nephron segment markers in heterozygous kidneys. Small boxes indicate the regions shown at high magnification in c. Scale bar, 1 mm. c Representative ECAD-positive and AQP1-, AQP2-, NCC-, and UMOD-negative cysts and AQP2-positive cysts in heterozygous kidneys. Asterisks indicate cysts. Scale bar, 100 µm. d Expressions of ECAD and PC1 in heterozygous kidneys. One representative PC1-negative cyst in Hetero #1 and two PC1-positive cysts in Hetero #3 are shown. Asterisks indicate cysts. Scale bar, 100 µm.