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Reviewed April 2010
What is the official name of the HADH gene?
The official name of this gene is “hydroxyacyl-CoA dehydrogenase.”
HADH is the gene's official symbol. The HADH gene is also known by other names, listed below.
Read more about gene names and symbols on the About page.
What is the normal function of the HADH gene?
The HADH gene provides instructions for making an enzyme called 3-hydroxyacyl-CoA dehydrogenase that is important for converting certain fats to energy. This enzyme is involved in a process called fatty acid oxidation, in which several enzymes work in a step-wise fashion to break down (metabolize) fats and convert them to energy. The role of 3-hydroxyacyl-CoA dehydrogenase is to metabolize groups of fats called medium-chain fatty acids and short-chain fatty acids. These fatty acids are found in foods such as milk and certain oils and are produced when larger fatty acids are metabolized.
3-hydroxyacyl-CoA dehydrogenase functions in mitochondria, the energy-producing centers within cells. This enzyme is especially important for the normal functioning of the heart, liver, kidneys, muscles, and pancreas. The pancreas makes enzymes that help digest food, and it also produces insulin, which controls how much sugar is passed from the blood into cells for conversion to energy.
3-hydroxyacyl-CoA dehydrogenase is essential in the process that converts medium-chain and short-chain fatty acids to ketones, the major source of energy used by the heart and muscles. During prolonged periods without food (fasting) or when energy demands are increased, ketones are also important for the liver and other tissues.
How are changes in the HADH gene related to health conditions?
Genetics Home Reference provides information about congenital hyperinsulinism, which is also associated with changes in the HADH gene.
Where is the HADH gene located?
Cytogenetic Location: 4q22-q26
Molecular Location on chromosome 4: base pairs 107,989,714 to 108,035,175
(Homo sapiens Annotation Release 107, GRCh38.p2) (
The HADH gene is located on the long (q) arm of chromosome 4 between positions 22 and 26.
More precisely, the HADH gene is located from base pair 107,989,714 to base pair 108,035,175 on chromosome 4.
See How do geneticists indicate the location of a gene? in the Handbook.
Where can I find additional information about HADH?
You and your healthcare professional may find the following resources about HADH helpful.
You may also be interested in these resources, which are designed for genetics professionals and researchers.
What other names do people use for the HADH gene or gene products?
See How are genetic conditions and genes named? in the Handbook.
Where can I find general information about genes?
The Handbook provides basic information about genetics in clear language.
These links provide additional genetics resources that may be useful.
What glossary definitions help with understanding HADH?
acids ; CoA ; coenzyme A ; congenital ; deficiency ; dehydrogenase ; enzyme ; familial ; fasting ; fatty acids ; gene ; hyperinsulinism ; hypoglycemia ; insulin ; lethargy ; mitochondria ; oxidation ; pancreas ; protein ; secretion
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (8 links)
The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? in the Handbook.