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The mitochondrial fatty acid oxidation (FAO) pathway is the primary mechanism by which fatty acids are broken down in mitochondria to generate energy, especially during fasting or when glucose is scarce. This process, also known as β-oxidation, involves a cyclic sequence of enzymatic reactions that convert fatty acids into acetyl-CoA, which then enters the tricarboxylic acid (TCA) cycle for further ATP production. Defects in any step can cause inherited metabolic disorders presenting with hypoketotic hypoglycemia, cardiomyopathy, arrhythmia or rhabdomyolysis—highlighting its critical physiological role. Accumulation of unmetabolized lipids due to impaired FAO contributes to insulin resistance.
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