HADHA Recombinant Rabbit Monoclonal Antibody [PSH01-29] (HA721653)

UNIPROT #

P40939

PROTEIN NAME

HADHA

SYNONYMS

3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit antibody;3 oxoacyl CoA thiolase antibody;78 kDa gastrin binding protein antibody;78 kDa gastrin-binding protein antibody;ECHA antibody;ECHA_HUMAN antibody;GBP antibody;HADH antibody;HADHA antibody;Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit antibody;LCEH antibody;LCHAD antibody;Long chain 3-hydroxyacyl-CoA dehydrogenase antibody;Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit antibody;Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunit antibody;Mitochondrial trifunctional enzyme alpha subunit antibody;Mitochondrial trifunctional protein alpha subunit antibody;MTPA antibody;Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit antibody;TP ALPHA antibody;TP-alpha antibody;Trifunctional enzyme subunit alpha mitochondrial precursor antibody

SUBCELLULAR LOCATION

Mitochondrion, Mitochondrion inner membrane.

FUNCTION

Trifunctional enzyme subunit alpha, mitochondrial also known as hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit is a protein that in humans is encoded by the HADHA gene. Mutations in HADHA have been associated with trifunctional protein deficiency or long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. 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 enzyme converts medium- and long-chain 2-enoyl-CoA compounds into the following 3-ketoacyl-CoA when NAD is solely present, and acetyl-CoA when NAD and CoASH are present. The alpha subunit catalyzes this reaction, and is attached to HADHB, which catalyzes the last step of the reaction.