Mitochondria function and dysfunction
Mitochondria: The Cell's Powerhouses One mechanism that may contribute to mitochondrial dysfunction is the accumulation of phytanic acid , a branched-chain fatty acid that can build up in certain inherited metabolic disorders, such as Refsum disease. Experimental studies have shown that high intracellular concentrations of phytanic acid can: Inhibit mitochondrial respiration Decrease ATP production Increase production of reactive oxygen species (ROS) Disrupt mitochondrial membrane function Could Phytanic Acid Affect Intracellular pH? Healthy cells use ATP-powered ion pumps—including the Na⁺/K⁺-ATPase , Ca²⁺-ATPases , and proton transport systems—to maintain intracellular pH and ionic balance. When mitochondrial ATP production declines: Less ATP is available to power these transporters. Proton handling becomes less efficient. Cellular ion gradients may become disrupted. Intracellular pH regulation may be impaired. Phytanic acid itself is amphipathic , meaning it possesses both hydr...