Hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha (HADHA)

Hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha (HADHA) is the alpha subunit of the mitochondrial trifunctional protein (TFP), a multienzyme complex critical for the mitochondrial beta-oxidation of long-chain fatty acids[1][4]. The protein is composed of 763 amino acids and, together with four alpha and four beta subunits, forms a complex that catalyzes the last three steps in the beta-oxidation cycle of fatty acids[1][4]. The alpha subunit catalyzes the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities, as well as participating in mitochondrial membrane phospholipid remodeling and interacting with mitochondrial respiratory chain complexes[1][4]. Mutations in HADHA cause trifunctional protein deficiency and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD), leading to severe metabolic decompensation, muscle, nerve, and cardiac involvement, and are managed primarily by metabolic and dietary interventions[4].

Other names

Trifunctional enzyme subunit alpha, mitochondrial3-hydroxyacyl-CoA dehydrogenase subunit alphaMitochondrial trifunctional protein, alpha subunitAlpha subunit of TFPLong-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), alpha subunitHydroxyacyl-CoA dehydrogenase, alpha subunitLCHAD (Alpha subunit)

Not applicable; drugs do not directly target this enzyme. Management strategies aim to bypass the metabolic block or prevent decompensation in deficiency states.