Fatty acid synthesisLong-chain l-3-hydroxyacyl CoA dehydrogenaseAlthough mitochondrial fatty acid beta-oxidation (FAO) is considered to be well understood, further elucidation of the pathway continues through evaluation of patients with FAO defects. The FAO pathway can be examined by measuring the 3-...
Fig. 3. Beta-oxidation of medium chain fatty acids which, unlike long chain fatty acids (LCFA), do not need to be activated by carnitine to access the inner mitochondrial membrane. CPT1- Carnitine Palmitoyl Transferase 1, CPT2- Carnitine Palmitoyl Transferase 2, CAC- Citric Acid Cycle, ATP...
Medium-chain acyl-CoA dehydrogenase (MCAD) is a mitochondrial flavoenzyme involved in mitochondrial fatty acid β-oxidation. MCAD catalyzes the first step in this process, which involves α and β-dehydrogenation of acyl-CoAs of medium chain lengths. It favors C6- to C12-CoA substrates and ha...
Medium-chain Acyl-CoA dehydrogenase deficiency (MCADD) is the most common inherited disorder of fatty acid beta-oxidation. Signs and symptoms of MCADD typically appear during infancy or early childhood and include vomiting, lethargy, and hypoglycemia. Pulmonary haemorrhage has previously been described ...
These properties have been used advantageously in the clinics for more than 50 years for treating lipid absorption disorders, undernourished patients, and more recently subjects with long-chain fatty acid oxidation defects. In these latter subjects, nutritional interventions with MCFA-containing ...
acylcarnitines; lymphocyte enzyme activity; medium-chain acyl-CoA dehydrogenase; newborn screening; fatty acid oxidation1. Introduction Medium-chain acyl-CoA dehydrogenase (MCAD, EC1.3.8.7) is a mitochondrial matrix flavoprotein essential for the beta-oxidation of medium-chain fatty acids, and its ...
chain fatty acids, which result from partial beta-oxidation. Their transconjugation from CoA to carnitine frees CoA for further use in beta-oxidation and tricarboxylic acid (Krebs) cycle reactions. This may be particularly important in the heart or skeletal muscle when short-chain beta-oxid...
As a feed additive, medium-chain fatty acids (MCFAs)/medium-chain fatty acid triglycerides (MCTs) have been used in ruminant production, but mostly added in the form of mixed esters. Studies have shown that MCTs may have a positive effect on feed intake or oxidative stress in animals, but...
As a feed additive, medium-chain fatty acids (MCFAs)/medium-chain fatty acid triglycerides (MCTs) have been used in ruminant production, but mostly added in the form of mixed esters. Studies have shown that MCTs may have a positive effect on feed intake
The fatty acid metabolism of extracellular glycolipids, mannosylerythritol lipids produced by the yeast Candida antarctica T-34 and used as biosurfactants, was examined using individual fatty compounds of various chain lengths (from C<SUB>12</SUB> to C<SUB>18</SUB>) as the sole source of carbo...