Anti-HADHB antibody

Supplementary info

Activity summary

The protein HADHB also called the beta subunit of the Mitochondrial Trifunctional Protein is a part of the enzyme system that breaks down fatty acids. It has an approximate molecular mass of 51 kDa. This protein performs mechanical functions that include enoyl-CoA hydratase hydroxyacyl-CoA dehydrogenase and thiolase activities which facilitate the conversion of fatty acids into acetyl-CoA units. HADHB is expressed in tissues with high energy demands especially in the liver heart and skeletal muscle where it plays an essential role in energy production.

Biological function summary

The HADHB protein forms a complex with HADHA creating the Mitochondrial Trifunctional Protein. This complex is involved in the inner mitochondrial membrane's beta-oxidation of long-chain fatty acids. Long-chain fatty acids serve as a vital energy source and the HADHB-HADHA complex makes their metabolism more efficient. This process provides ATP essential for maintaining cellular energy homeostasis especially during fasting and intense exercise.

Pathways

HADHB participates in the fatty acid beta-oxidation pathway a critical component of lipid metabolism. Through this pathway HADHB interacts with proteins like ACADVL and ACADM which catalyze different stages of fatty acid breakdown. Beta-oxidation plays an important role in producing acetyl-CoA subsequently entering the citric acid cycle linking HADHB to energy metabolism. These interactions emphasize the importance of HADHB in maintaining metabolic balance within cells.

Associated diseases and disorders

HADHB mutations can lead to mitochondrial trifunctional protein deficiency which results in metabolic disorders like fatty acid oxidation disorders and peripheral neuropathy. These conditions impair the body's ability to break down fatty acids affecting energy homeostasis. Disorders related to HADHB may also involve proteins such as CPT1A which transfers fatty acids into mitochondria highlighting the interconnectedness of these metabolic pathways. Understanding HADHB's role in these diseases enhances the potential for targeted therapeutic strategies.