Function
In the process of energy production from fats, ACAA2 plays a critical role as one of the enzymes that catalyzes the final step of the mitochondrial beta-oxidation pathway. This aerobic process breaks down fatty acids into acetyl-CoA. Utilizing free coenzyme A/CoA, ACAA2 catalyzes the thiolytic cleavage of medium- to long-chain unbranched 3-oxoacyl-CoAs, yielding acetyl-CoA and a fatty acyl-CoA shortened by two carbon atoms. Additionally, it catalyzes the condensation of two acetyl-CoA molecules into acetoacetyl-CoA, potentially contributing to the production of ketone bodies. ACAA2 also exhibits hydrolase activity on various fatty acyl-CoAs, suggesting a possible role in acetate production as a side reaction to beta-oxidation. Notably, ACAA2 inhibits BNIP3-mediated apoptosis and mitochondrial damage.