Morphological change of mitochondria in ADPKD animal models. (A) Electron microscope image of 7-day-old Ksp-Cre PKD1flox/+ mice and Ksp-Cre PKD1flox/flox mice. Left, distal tubules of Ksp-Cre PKD1flox/+ mice. Middle, cyst epithelial cells from Ksp-Cre PKD1flox/+ mice; right, higher magnification of the middle panel. Arrows show normal mitochondria, and arrowheads show mitochondria that became swollen with indistinct cristae. *, cystic cavity. (B) Electron microscope images of proximal tubules from the kidneys of controls (+/+) and cysts derived from the proximal tubules of kidneys from Cy/+ rats. The mitochondria of cyst-lining cells from Cy/+ kidneys were fragmented (arrow). *, cystic cavity.