title: Acylcoenzyme A dehydrogenase deficiency in heart tissue from infants who died unexpectedly with fatty change in the liver.
abstract: Heart muscle from infants who died unexpectedly and who showed fatty changes in the liver at necropsy was analysed for long chain and medium chain acylcoenzyme A dehydrogenase activities by using the natural electron acceptor. In two of the seven cases investigated a deficiency in acylcoenzyme A dehydrogenase activity was found. In one case the deficiency was in medium chain acylcoenzyme A dehydrogenase activity and in the other long chain acylcoenzyme A dehydrogenase activity. These findings emphasise the importance of investigating fatty acid oxidation in infants who have died unexpectedly.
mesh_terms: D042964:Acyl-CoA Dehydrogenase; D044943:Fatty Acid Desaturases / Q000172:deficiency*; D005227:Fatty Acids / Q000032:analysis; D005234:Fatty Liver / Q000150:complications / Q000378:metabolism; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000032:analysis; D008297:Male; D009206:Myocardium / Q000201:enzymology*; D012189:Retrospective Studies; D013398:Sudden Infant Death / Q000201:enzymology* / Q000378:metabolism
chemical_list: D005227:Fatty Acids; D044943:Fatty Acid Desaturases; D042964:Acyl-CoA Dehydrogenase