The carnitine palmitoyl transferase (CPT) system is crucial for mitochondrial β-oxidation of long-chain fatty acids (Table 3).200 Overexpression of CPT1c in the mouse brain caused microencephaly, and CPT1c-KO mice demonstrated a marked reduction in spatial learning ability201,202 and increased sensitivity to oxidative stress.203 In addition, genetic or pharmacological inhibition of acetyl-CoA acetyltransferase 1 (ACAT), an enzyme that catalyzes the final step in the mitochondrial beta-oxidation pathway, is thought to exert an inhibitory effect on the brain lesion process.204–207. The gene discussed is ACAT1; the disease is microcephaly.