Protein Summary

This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.

Alternative names and synonyms associated with Hydroxyacyl-Coenzyme A Dehydrogenase/3-Ketoacyl-Coenzyme A Thiolase/enoyl-Coenzyme A Hydratase (Trifunctional Protein), alpha Subunit (HADHA)

• hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha (HADHA) antibody
• hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha (hadha) antibody
• hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit (HADHA) antibody
• hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha S homeolog (hadha.S) antibody
• hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha a (hadhaa) antibody
• Show all gene names
• hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit (Hadha) antibody
• hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit (Hadha) antibody

• C77020 antibody
• cb1055 antibody
• ECHA antibody
• GBP antibody
• HADH antibody
• Show all synonym aliases
• hadha antibody
• im:7136067 antibody
• LCEH antibody
• LCHAD antibody
• Mtpa antibody
• RGD1560655 antibody
• TP-ALPHA antibody
• wu:fa14c08 antibody

Protein level used designations for HADHA

trifunctional enzyme subunit alpha, mitochondrial , dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit hydroxyacyl-Coenzyme A , hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit , mitochondrial trifunctional protein, alpha subunit , CFR-associated protein p70 , hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein) , hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit , hydroxyacyl-coenzyme A dehydrogenase/3-ketoacyl-coenzyme A thiolase/enoyl-coenzyme A hydratase (trifunctional protein) alpha subunit , mitochondrial trifunctional protein, alpha subunit-like , trifunctional enzyme subunit alpha, mitochondrial-like , 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit , 3-oxoacyl-CoA thiolase , 78 kDa gastrin-binding protein , gastrin-binding protein , long-chain 2-enoyl-CoA hydratase , long-chain-3-hydroxyacyl-CoA dehydrogenase , mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit , mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit , mitochondrial trifunctional enzyme, alpha subunit , TP-alpha