The HADHA 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 alpha subunits contain two of the enzymes, known as long-chain 3-hydroxyacyl-CoA dehydrogenase and long-chain 2-enoyl-CoA hydratase. The beta subunits contain the third enzyme. 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.
Other Names
ECHA_HUMAN; GBP; hydroxyacyl dehydrogenase, subunit A; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; LCEH; LCHAD; long-chain hydroxyacyl-CoA dehydrogenase; mitochondrial trifunctional protein, alpha subunit; MTPA
Related Genetics Topics
- Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency — MedlinePlus Genetics
- Mitochondrial trifunctional protein deficiency — MedlinePlus Genetics
Identifiers
- NCBI Gene: 3030
- OMIM: 600890
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
