HADHA Products

Description

HADHA 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.

Bioinformatics

Entrez	3030 Human
Uniprot	NP_000173 Human NP_000173.2 Human P40939 Human
Product By Gene ID	3030
Alternate Names	3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit 3-oxoacyl-CoA thiolase 78 kDa gastrin-binding protein ECHA gastrin-binding protein GBP HADH hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase(trifunctional protein), alpha subunit hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme Athiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit LCEH LCHAD long-chain 2-enoyl-CoA hydratase long-chain-3-hydroxyacyl-CoA dehydrogenase MGC1728 mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alphasubunit mitochondrial trifunctional enzyme, alpha subunit mitochondrial trifunctional protein, alpha subunit MTPATP-ALPHA TP-alpha trifunctional enzyme subunit alpha, mitochondrial