HADHB : hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta
Description
The HADHB (hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta) is a protein-coding gene located on chromosome 2.
The HADHB gene provides instructions for making part of an enzyme complex called mitochondrial trifunctional protein. This enzyme complex functions in mitochondria, the energy-producing centers within cells. Mitochondrial trifunctional protein is made of eight parts (subunits). Four alpha subunits are produced from the HADHA gene, and four beta subunits are produced from the HADHB gene. As the name suggests, mitochondrial trifunctional protein contains three enzymes that each perform a different function. The beta subunits contain one of the enzymes, known as long-chain 3-keto-acyl-CoA thiolase. The alpha subunits contain the other two enzymes. These enzymes are essential for fatty acid oxidation, which is the multistep process that breaks down (metabolizes) fats and converts them to energy.Mitochondrial trifunctional protein is required to metabolize a group of fats called long-chain fatty acids. Long-chain fatty acids are found in foods such as milk and certain oils. These fatty acids are stored in the body's fat tissues. Fatty acids are a major source of energy for the heart and muscles. During periods of fasting, fatty acids are also an important energy source for the liver and other tissues.
The HADHB gene encodes the beta subunit of the mitochondrial trifunctional protein (MTP), which is a heterotetrameric complex composed of two alpha (HADHA) and two beta (HADHB) subunits. MTP catalyzes the last three reactions of the mitochondrial beta-oxidation pathway, a major energy-producing process in tissues that breaks down fatty acids into acetyl-CoA. The beta subunit specifically carries the 3-ketoacyl-CoA thiolase activity, which is essential for the breakdown of long-chain fatty acids. MTP exhibits specificity for long-chain fatty acids.
HADHB is also known as ECHB, MSTP029, MTPB, MTPD, MTPD2, TP-BETA.
