Mitochondrial fatty acid transport and oxidation pathway

The **mitochondrial fatty acid transport and oxidation pathway** refers to the series of biochemical processes by which long-chain fatty acids are transported into mitochondria and subsequently broken down via beta‑oxidation to generate acetyl-CoA, NADH, and FADH₂. These products feed into the tricarboxylic acid cycle and electron transport chain for ATP production. The process involves several key steps: 1. **Activation:** Fatty acids are activated in the cytosol by conjugation with coenzyme A to form acyl-CoA. 2. **Transport:** Long-chain acyl-CoA cannot cross mitochondrial membranes directly; instead, it is converted by carnitine palmitoyltransferase I (CPT1) into acylcarnitine for translocation across the inner membrane via carnitine translocase. Carnitine palmitoyltransferase II (CPT2) then reconverts it back to acyl-CoA inside the matrix[1][3][6]. 3. **Beta-Oxidation:** Acyl-CoA undergoes four recurring enzymatic reactions—dehydrogenation by acyl-CoA dehydrogenase, hydration by enoyl-CoA hydratase, another dehydrogenation by hydroxyacyl-CoA dehydrogenase, and thiolytic cleavage by thiolase—producing acetyl‑CoA units that enter further metabolic cycles[1][4][5]. This process is essential for energy homeostasis in tissues with high energy demands such as heart muscle[1]. Defects at any step can result in metabolic diseases. **Note:** This entry describes an entire *metabolic pathway*, not a discrete molecular target such as an enzyme or receptor. Therefore, *is_target* should be set to false, and *is_incorrect* should be set to true because "Mitochondrial fatty acid transport/oxidation pathway" does not refer to a single canonical druggable target but rather encompasses multiple proteins including CPT1/2, CAT/translocases, various dehydrogenases etc.[3][4].

Other names

Fatty acid β-oxidation pathwayMitochondrial fatty acid oxidationBeta-oxidation of fatty acidsFatty acid mitochondrial import and oxidation