Chapter 5 Oxidation of fatty acids in eukaryotes

Abstract

Publisher Summary This chapter discusses the pathway of β-oxidation, uptake and activation of fatty acids in animal cells, fatty acid oxidation in mitochondria, fatty acid oxidation in peroxisomes, and inherited diseases of fatty acid oxidation. In animal cells, fatty acids are degraded in both mitochondria and peroxisomes, whereas in lower eukaryotes β-oxidation is confined to peroxisomes. Mitochondrial β-oxidation provides energy for oxidative phosphorylation and generates acetyl- coenzyme A (CoA) for ketogenesis in liver. The enzymes of β-oxidation either are associated with the inner mitochondrial membrane or are located in the mitochondrial matrix. The four acyl-CoA dehydrogenases, including short-chain, medium-chain, long-chain, and very-long-chain acyl-CoA dehydrogenases, assure a complete degradation of all fatty acids that can be metabolized by mitochondrial β-oxidation. The molecular characterization of most β-oxidation enzymes has yielded a wealth of structural information while the dynamics of this pathway remain less well understood. The cooperation between peroxisomes and mitochondria in fatty acid oxidation also remains to be studied.