Medium Chain Acyl CoA
Dehydrogenase Deficiency (MCADD) can be easily managed once diagnosed but often the first sign is when the sufferer is taken seriously ill.
Normal functioning of the CPT system is required to ensure long chain fatty acyl CoA
is transported to the mitochondrial matrix where it is needed for [beta]-oxidation.
Steady-state levels of acyl CoA
oxidase (ACO); 3-hydroxy, 3-methylglutaryl CoA reductase (HMG-R); and apolipoprotein A-I (Apo A-I) mRNA were examined after 24-hour incubation in the absence (control) or presence of 100 [micro]m each of linoleic acid (LA), cis-9, traps-11 CLA or traps-10, cis-12 CLA.
Molecular cloning of the peroxisome proliferators-induced 46kDa cytosolic acyl CoA
thioesterase from mouse and rat liver--recombinant expression in Escherichia coil, tissue expression and nutritional regulation.
The major diagnoses were fatty acid oxidation defects, particularly medium-chain acyl CoA
Well-known mitochondrial diseases of children are Leigh's syndrome, cardiomyopathty, and medium-chain acyl CoA
dehydrogenase (MCAD) deficiency Typical mitochondrial diseases of young adults include Leber's hereditary optic neuropathy and Kearns-Sayre syndrome.