Alterations in mitochondrial metabolism in ADPKD-cyst-derived cells with a homozygous PKD1 mutation. (A) Measurement of the mitochondrial OCR of normal tubular cells derived from distal tubules (RCTEC-DBA) and cyst-derived cells with a PKD1 homozygous mutation derived from distal tubules (WT 9-12). (B) Seahorse XF Cell Mito stress test profile of the key parameters of mitochondrial respiration. (C to H) Basal respiration (C), ATP production with mitochondrial respiration (D), mitochondrial respiration and spare capacity (E and F, respectively), proton leakage from mitochondria (G), and nonmitochondrial respiration (H) in WT 9-12 compared to RCTEC-DBA. Each group, n = 30. Results represent the means ± standard deviations. ***, P < 0.001.