Notably, increased nonmitochondrial respiration was also found in the cells with the heterozygous PKD1 mutation (Fig. 8G), as was found in the cells with the homozygous PKD1 mutation; these data suggest that not only mitochondrial superoxide but also nonmitochondrial ROS will increase in cyst-derived cells compared with normal tubular cells. Here, PKD1 is linked to cyst.