HADHA (E-8) Anticorpo: sc-374497

HADHA Background Information
HADHA (Trifunctional enzyme subunit alpha, mitochondrial), also known as TP-alpha, is the 763 amino acid alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. This mitochondrial complex is complosed of four alpha (HADHA) and four beta (HADHB) subunits, and the alpha subunit (HADHA) is responsible for catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in the HADHA gene can lead to long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency or mitochondrial trifunctional protein deficiency. LCHAD deficiency is characterized by a deficiency of the dehydrogenase activity with normal hydratase activity and moderately decreased thiolase activity. In mitochondrial trifunctional protein deficiency, all three activities of the protein, dehydrogenase, hydratase, and thiolase, are deficient.