HADHB Human
Description
Product Specs
Product Science Overview
2-Enoyl-Coenzyme A (CoA) Hydratase, Beta, also known as HADHB, is an enzyme that plays a crucial role in the metabolism of fatty acids. This enzyme is part of the mitochondrial trifunctional protein complex, which is involved in the beta-oxidation pathway of fatty acid metabolism. The human recombinant form of this enzyme is produced using recombinant DNA technology, which allows for the production of large quantities of the enzyme for research and therapeutic purposes.
HADHB is a non-glycosylated polypeptide chain consisting of 464 amino acids and has a molecular mass of approximately 49.9 kDa . The enzyme is fused to a 23 amino acid His-tag at the N-terminus, which facilitates its purification through chromatographic techniques .
The primary function of HADHB is to catalyze the hydration of 2-trans-enoyl-CoA to L-3-hydroxyacyl-CoA, a critical step in the beta-oxidation of fatty acids . This reaction involves the addition of a hydroxyl group and a proton to the unsaturated beta-carbon on a fatty-acyl CoA, converting it into a beta-hydroxy acyl-CoA . The enzyme’s efficiency is attributed to its hexameric structure, which provides six active sites for catalysis .
HADHB is essential for the efficient metabolism of fatty acids, which are a significant source of energy for the human body. The beta-oxidation pathway, in which HADHB is involved, breaks down long-chain fatty acids into acetyl-CoA units. These units then enter the citric acid cycle (Krebs cycle) to produce ATP, the primary energy currency of the cell .
In addition to its role in energy production, HADHB is also involved in the metabolism of branched-chain amino acids, such as leucine . This enzyme’s activity is crucial for maintaining energy homeostasis, especially during periods of fasting or prolonged exercise when fatty acids become the primary energy source.
Mutations in the HADHB gene can lead to metabolic disorders, such as mitochondrial trifunctional protein deficiency (MTPD) and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD). These disorders are characterized by the accumulation of fatty acids and their derivatives, leading to symptoms such as hypoglycemia, muscle weakness, and cardiomyopathy .
Recombinant HADHB is used in research to study these metabolic disorders and to develop potential therapeutic interventions. The availability of human recombinant HADHB allows for detailed biochemical and structural studies, which are essential for understanding the enzyme’s function and its role in disease.
