Carnitine acetyltransferase is a mitochondrial enzyme essential for fuel utilization. It is present in the mitochondrial matrix and peroxisome. It is involved in the fatty acid metabolism. It facilitates conversion of acetyl-CoA and carnitine to acetylcarnitine and CoA respectively. This conversion facilitates peroxisomal β-oxidation by importing acetyl group for mitochondrial oxidation.
Carnitine acetyltransferase maintains the cellular and mitochondrial levels of acetyl-CoA, a key cofactor required for oxidative metabolism, by catalyzing an equilibrium between acetyl-CoA and acetyl-L-carnitine, a storage form of activated acetate. Carnitine acetyltransferase also maintains the pool of acetyl-CoA required for neuronal and nonneuronal acetylcholine production.