Human 3-Ketoacyl-CoA Thiolase, Mitochondrial (ACAA2) Protein

Background

Acetyl Coenzyme A Acyltransferase 2 (ACAA2), also known as mitochondrial 3-ketoacyl-CoA thiolase, is an essential mitochondrial enzyme involved in the beta-oxidation of fatty acids. It catalyzes the thiolytic cleavage of 3-ketoacyl-CoA into acetyl-CoA, which then enters the TCA cycle for ATP production. ACAA2 is highly expressed in energy-demanding tissues such as the liver, heart, and muscle, where it plays a key role in maintaining metabolic flexibility and energy production during fasting or exercise. Mutations or deficiencies in ACAA2 can impair mitochondrial fatty acid oxidation, leading to conditions such as mitochondrial fatty acid oxidation disorders and myopathies. Beyond its metabolic role, ACAA2 activity influences reactive oxygen species (ROS) production and mitochondrial dynamics, linking it to cellular stress responses and metabolic regulation. Ongoing research explores its potential as a therapeutic target for metabolic diseases and mitochondrial dysfunction.