Acylcoenzyme A dehydrogenase deficiency in heart tissue from infants who died unexpectedly with fatty change in the liver.
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.
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
D005227:Fatty Acids; D044943:Fatty Acid Desaturases; D042964:Acyl-CoA Dehydrogenase