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Long-chain 3-ketoacyl-CoA thiolase (LCKAT) is a mitochondrial enzyme that plays a critical role in the β-oxidation of long-chain fatty acids. It catalyzes the final step in this pathway, cleaving 3-ketoacyl-CoA into acetyl-CoA and a shortened acyl-CoA molecule. LCKAT is part of the mitochondrial trifunctional protein (MTP). Deficiency or mutations affecting LCKAT result in impaired oxidation of long-chain fatty acids, leading to severe metabolic disorders.
Inhibition of long-chain 3-ketoacyl-CoA thiolase activity
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