MAMMALIAN MITOCHONDRIAL BETA-OXIDATION

The enzymic stages of mammalian mitochondrial beta-oxidation were eluc idated some 30-40 years ago. However, the discovery of a membrane-asso ciated multifunctional enzyme of beta-oxidation, a membrane-associated acyl-CoA dehydrogenase and characterization of the carnitine palmitoy l transferase system at the protein and at the genetic level has demon strated that the enzymes of the system itself are incompletely underst ood. Deficiencies of many of the enzymes have been recognized as impor tant causes of disease. In addition, the study of these disorders has led to a greater understanding of the molecular mechanism of beta-oxid ation and the import, processing and assembly of the beta-oxidation en zymes within the mitochondrion. The tissue-specific regulation, intram itochondrial control and supramolecular organization of the pathway is becoming better understood as sensitive analytical and molecular tech niques are applied. This review aims to cover enzymological and organi zational aspects of mitochondrial beta-oxidation together with the bio chemical aspects of inherited disorders of beta-oxidation and the intr insic control of beta-oxidation.