Mitochondrial trifunctional protein

Mitochondrial trifunctional protein is a protein which catalyzes several reactions in beta oxidation. It has two subunits:


 * HADHA
 * HADHB

The three functions are long-chain 3-hydroxy acyl-coenzyme A dehydrogenase, 2-enoyl coenzyme A (CoA) hydratase, and long-chain 3-ketoacyl CoA thiolase.

Pathology
Disorders are associated with:


 * Mitochondrial trifunctional protein deficiency
 * LCHAD deficiency