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Truncating mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene increase extracellular acidification rate (ECAR) and oxygen consumption rate (OCR). A: PKHD1 truncating mutations increased nonglycolytic acidification rate in human embryonic kidney (HEK-293) cells (P = 0.002). B: PKHD1 truncating mutations increased basal OCR (P = 0.015). C: PKHD1 truncating mutations increased ATP-linked OCR (P = 0.004).
