A Defect in the Transport of Long-Chain Fatty Acids Associated with Acute Liver Failure

Abstract

Fatty-acid oxidation has a major role in energy production during periods of fasting. When body glucose is depleted, fatty acids are mobilized from adipose tissue, taken up by the liver, and converted to ketone bodies, a major alternative source of energy for peripheral tissues.1 At the cellular level, after being transported through the cell membrane and then into the mitochondria by means of a carnitine-dependent system, long-chain fatty acids are predominantly oxidized in mitochondria.2,3 Common clinical features of disorders of fatty-acid oxidation are metabolic decompensation during fasting, hypoketotic hypoglycemia, and acute dysfunction of fatty-acid–dependent tissues (skeletal muscle, heart, and . . .