Hydroxyacyl-Coenzyme A dehydrogenase
Encyclopedia
Hydroxyacyl-Coenzyme A dehydrogenase also known as HADH is an enzyme
which in humans is encoded by the HADH gene
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gene family. The encoded protein functions in the mitochondrial matrix
to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids.
. A deficiency is associated with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency
.
Enzyme
Enzymes are proteins that catalyze chemical reactions. In enzymatic reactions, the molecules at the beginning of the process, called substrates, are converted into different molecules, called products. Almost all chemical reactions in a biological cell need enzymes in order to occur at rates...
which in humans is encoded by the HADH gene
Gene
A gene is a molecular unit of heredity of a living organism. It is a name given to some stretches of DNA and RNA that code for a type of protein or for an RNA chain that has a function in the organism. Living beings depend on genes, as they specify all proteins and functional RNA chains...
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Function
This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase3-hydroxyacyl-CoA dehydrogenase
In enzymology, a 3-hydroxyacyl-CoA dehydrogenase is an enzyme that catalyzes the chemical reaction-3-hydroxyacyl-CoA + NAD+ \rightleftharpoons 3-oxoacyl-CoA + NADH + H+...
gene family. The encoded protein functions in the mitochondrial matrix
Mitochondrial matrix
In the mitochondrion, the matrix contains soluble enzymes that catalyze the oxidation of pyruvate and other small organic molecules.The mitochondrial matrix also contains the mitochondria's DNA and ribosomes. The word "matrix" stems from the fact that this space is viscous, compared to the...
to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids.
Clinical significance
Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemiaHyperinsulinemic hypoglycemia
Hyperinsulinemic hypoglycemia describes the condition and effects of low blood glucose caused by excessive insulin. Hypoglycemia due to excess insulin is the most common type of serious hypoglycemia. It can be due to endogenous or injected insulin.-Causes:...
. A deficiency is associated with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency
3-hydroxyacyl-coenzyme A dehydrogenase deficiency
3-hydroxyacyl-coenzyme A dehydrogenase deficiency is a rare condition that prevents the body from converting certain fats to energy, particularly during fasting. Normally, through a process called fatty acid oxidation, several enzymes work in a step-wise fashion to metabolize fats and convert them...
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