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The mitochondrial beta oxidation pathway is a central metabolic process responsible for the breakdown of fatty acids to generate energy, primarily in the form of ATP. This pathway occurs within the mitochondria of eukaryotic cells and is especially active in tissues with high energy demands such as heart, skeletal muscle, and liver. It involves a series of enzymatic steps that sequentially shorten fatty acid chains, producing acetyl-CoA, NADH, and FADH2, which are then used for ATP production. Defects in this pathway can lead to various metabolic disorders.
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