Abnormality of Odd-Numbered Long-Chain Fatty Acids in Erythrocyte Membrane Lipids from Patients with Disorders of Propionate Metabolism

摘要

In propionic acidemia and methylmalonic aciduria, disorders of propionate catabolism, excess intracellular propionyl-CoA leads to an enhanced synthesis of odd-numbered long-chain fatty acids. Red cell membrane lipids of patients with these disorders contain pentadecanoic, heptadecanoic, and heptadecenoic acids in excess of normal levels. The odd-numbered long-chain fatty acid content may reflect individual differences in severity of these disorders as well as the degree of effective dietary control. It might be a useful long term parameter for determining the effectiveness of clinical management.