The Association Between Acute Fatty Liver of Pregnancy and Fatty Acid Oxidation Disorders  Patricia A. Jamerson  Journal of Obstetric, Gynecologic & Neonatal Nursing  Volume 34, Issue 1, Pages 87-92 (January 2005) DOI: 10.1177/0884217504272800 Copyright © 2005 AWHONN, the Association of Women's Health, Obstetric and Neonatal Nurses Terms and Conditions

Figure 1 The metabolism of fatty acids through b-oxidation is a repetitive process that occurs in the mitochondrial matrix until all that remains is a two-carbon fatty acid. Each pass through b-oxidation shortens the fatty acid by two carbons. However, transport across the mitochondrial membrane and into the matrix is regulated. Fatty acids must first undergo a series of reactions before entering the matrix. First, fatty acids in the cytoplasm are activated by acyl-CoA synthetase to form acyl-CoA esters. The acyl-CoA esters are then facilitated across the mitochondrial membrane by reacting with carnitine. This process, called the carnitine cycle, is a two-step process. First, acyl-CoA is converted to acylcarnitine by carnitine palmitoyltransferase (CPT) I enzyme, located on the outer mitochondrial membrane. Next, acylcar-nitine is re-esterified to acyl-CoA by CPT II, which is located on the matrix side of the inner mitochondrial membrane and transported into the mitochondria by carnitine-acylcarnitine translocase. Once inside the matrix, the four-step b-oxidation process occurs. First, acyl-CoA, in a dehydrogenation process, is catalyzed by size-specific (long, medium, short) acyl-CoA dehydrogenase to form 2,3-enoyl-CoA. Next, 2,3-enoyl-CoA is hydrated to form 3-L-hydroxyacyl-CoA. This step is catalyzed by enoyl-CoA-hydrase and size-specific acyl-CoA dehydrogenase enzymes. Then, 3-L-hydroxyacyl-CoA is dehydrogenated in a reaction catalyzed by 3-L-hydroxyacyl-CoA dehydrogenase to form 3-ketoacyl-CoA, which in the final step undergoes cleavage catalyzed by 3-ketoa-cyl-CoA thiolase to form acetyl-CoA. Acetyl-CoA can either enter the Krebs tricarboxylic acid cycle or be used to make ketone bodies. Journal of Obstetric, Gynecologic & Neonatal Nursing 2005 34, 87-92DOI: (10.1177/0884217504272800) Copyright © 2005 AWHONN, the Association of Women's Health, Obstetric and Neonatal Nurses Terms and Conditions