An NAD-dependent 3-hydroxyacyl CoA dehydrogenase that has specificity for acyl chains containing 8 and 10 carbons.

A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. It contains enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, and acetyl-CoA C-acyltransferase activities and plays an important role in the metabolism of long chain FATTY ACIDS.

Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in the presence of NAD. They are key enzymes in the oxidation of fatty acids and in mitochondrial fatty acid synthesis.

The alpha subunit of mitochondrial trifunctional protein. It contains both enoyl-CoA hydratase activity (EC 4.2.1.17) and long-chain-3-hydroxyacyl-CoA dehydrogenase activity (EC 1.1.1.211). There are four of these alpha subunits in each mitochondrial trifunctional protein molecule.

Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.

Systems of enzymes which function sequentially by catalyzing consecutive reactions linked by common metabolic intermediates. They may involve simply a transfer of water molecules or hydrogen atoms and may be associated with large supramolecular structures such as MITOCHONDRIA or RIBOSOMES.