Abnormality of odd-numbered long-chain fatty acids in erythrocyte membrane lipids from patients with disorders of propionate metabolism

Abstract

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. © 1989 International Pediatric Research Foundation, Inc.