Acetyl-CoA acyltransferase 2 (ACAA2)

Acetyl-CoA acyltransferase 2 (ACAA2) is a mitochondrial enzyme that catalyzes the final step of the fatty acid beta-oxidation pathway, converting 3-oxoacyl-CoA to acetyl-CoA and shortened acyl-CoA, which is essential for cellular energy production from fatty acids[1][2][3]. ACAA2 also displays hydrolase activity and participates in ketone body synthesis. Beyond its metabolic role, ACAA2 interacts with apoptosis-related proteins like BNIP3, linking fatty acid metabolism with cell survival and death pathways[1][3][5]. Dysregulation or altered expression of ACAA2 has been connected with metabolic diseases, cancer cell metabolism, cardiovascular risk, drug-induced liver injury, and modulated function during viral infections[1][2][3]. No direct disease-causing mutations have been reported, but the gene locus is associated with variations in blood lipids and disease risk[1][2]. While not yet an established therapeutic target for drugs in clinical use, ACAA2 inhibitors are under research for possible metabolic interventions[4].

Other names

3-ketoacyl-CoA thiolase, mitochondrialAcetyl-Coenzyme A acyltransferase 2Mitochondrial 3-oxoacyl-CoA thiolaseAcyl-CoA hydrolase, mitochondrialAcetyl-CoA acetyltransferaseDSAECBeta-ketothiolaseT1EC 2.3.1.16[1][2]

Inhibition of ACAA2 disrupts mitochondrial fatty acid beta-oxidation, leading to reduced energy production from fatty acids[4].