The epidemiology of medium chain acyl-CoA
dehydrogenase deficiency: An update.
Inactivation of General Acyl-CoA
Dehydrogenase from Pig Kidney by a Metabolite of Hypoglycin A.
Whether the reaction is anabolic or catabolic, the essential substrate, fatty acyl-CoA
, is catalyzed by LACS.
Ethylmalonic aciduria is also associated with several other conditions, including short-chain acyl-CoA
dehydrogenase deficiency, multiple acyl-CoA
dehydrogenase deficiency, and Jamaican vomiting sickness.
Medium chain acyl-CoA
dehydrogenase (MCAD) is involved in fatty acid oxidation, and it is regulated by AMPK .
Clinical features and ETFDH mutation spectrum in a cohort of 90 Chinese patients with late-onset multiple acyl-CoA
Additionally, an increase in the enzymes present in the metabolism of fatty acids, such as Acetyl-CoA Synthetase (ACeCS1) and Acyl-CoA
Synthetase (ACSL1), and an induction of oxidative stress were observed.
Hansen, "Role of acyl-CoA
binding protein in acyl-CoA
metabolism and acyl-CoA-mediated cell signaling," Journal of Nutrition, vol.
MCAD deficiency is inherited as an autosomal recessive trait and is caused by mutations in the medium-chain acyl-CoA
dehydrogenase (ACADM) gene, which is located on chromosome 1p31 and consists of 12 exons spanning 44 Kb .
Caffeic acid and chlorogenic acid significantly inhibited fatty acid synthase, 3-hydroxy-3-methylglutaryl CoA reductase and acyl-CoA
:cholesterol acyltransferase activities, while they increased fatty acid beta-oxidation activity and peroxisome proliferator-activated receptors alpha expression in the liver compared to the high-fat group.
Those babies who have their newborn bloodspot sample from this month will be offered screening for medium chain acyl-CoA
dehydrogenase deficiency (MCADD).