Abnormality of odd-numbered long-chain fatty acids in erythrocyte membrane lipids from patients with disorders of propionate metabolism.
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.
Wendel
U
U
Kinderklinik, Universität Düsseldorf, Federal Republic of Germany.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004910:Erythrocyte Membrane / Q000032:analysis*; D005227:Fatty Acids / Q000032:analysis*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008314:Malonates / Q000652:urine*; D008563:Membrane Lipids / Q000032:analysis*; D008661:Metabolism, Inborn Errors / Q000097:blood*; D008764:Methylmalonic Acid / Q000652:urine*; D010713:Phosphatidylcholines / Q000032:analysis; D010714:Phosphatidylethanolamines / Q000032:analysis; D011422:Propionates / Q000097:blood* / Q000378:metabolism
D005227:Fatty Acids; D008314:Malonates; D008563:Membrane Lipids; D010713:Phosphatidylcholines; D010714:Phosphatidylethanolamines; D011422:Propionates; D008764:Methylmalonic Acid; C029658:propionic acid