CPT1A

CPT1A is an enzyme encoded by the CPT1A gene, which is crucial for fatty acid metabolism. It is located in the outer mitochondrial membrane and is involved in the transport of long-chain fatty acids into the mitochondria, where they undergo β-oxidation to generate energy. CPT1A catalyzes the conversion of fatty acyl-CoA to acylcarnitine, a necessary step for fatty acid entry into the mitochondria. Mutations in the CPT1A gene can lead to metabolic disorders, such as CPT1 deficiency, which impairs the ability to oxidize fatty acids, leading to hypoglycemia and other metabolic issues, particularly during fasting or prolonged exercise.