Acetyl Coenzyme A Acyltransferase 2 (ACAA2)

Mammalian tissues have 5 known types of thiolase: ACAT2, which is involved in cholesterol biosynthesis, peroxisomal ACAA1, which is involved in beta-oxidation, mitochondrial ACAT1, which appears to function in ketone body metabolism, mitochondrial ACAA2, which catalyzes the last step of the mitochondrial fatty acid beta-oxidation spiral, and mitochondrial trifunctional protein, which also catalyzes the last step of the mitochondrial fatty acid beta-oxidation spiral. The deduced 397-amino acid ACAA2 protein contains a putative 16-amino acid N-terminal mitochondrial targeting signal. Human and rat ACAA2 share 86.6% amino acid sequence identity. Northern blot analysis found a 1.6-kb ACAA2 transcript in human liver, fibroblasts, and intercostal muscle; ACAA2 expression was barely detectable in spleen and kidney.