2-hydroxyglutarate dehydrogenase
Encyclopedia
In enzymology, a 2-hydroxyglutarate dehydrogenase is an enzyme
that catalyzes
the chemical reaction
-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
Thus, the two substrates
of this enzyme are (S)-2-hydroxyglutarate
and acceptor
, whereas its two products
are 2-oxoglutarate and reduced acceptor. However, enzymes with specificity to (R)-2-hydroxyglutarate dehydrogenase also exist in both mammals and plants even though they have not been given a unique EC number. This enzyme participates in butanoate metabolism.
s, specifically those acting on the CH-OH group of donor with other acceptors. The systematic name of this enzyme class is (S)-2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:
) or in the model plant Arabidopsis thaliana
leads to massive accumulation of D-2-hydroxyglutarate
. In humans this results in the fatal neurometabolic disorder 2-Hydroxyglutaric aciduria whereas plants seem to be to a large extent unaffected by high cellular concentrations of this compound.
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...
that catalyzes
Catalysis
Catalysis is the change in rate of a chemical reaction due to the participation of a substance called a catalyst. Unlike other reagents that participate in the chemical reaction, a catalyst is not consumed by the reaction itself. A catalyst may participate in multiple chemical transformations....
the chemical reaction
Chemical reaction
A chemical reaction is a process that leads to the transformation of one set of chemical substances to another. Chemical reactions can be either spontaneous, requiring no input of energy, or non-spontaneous, typically following the input of some type of energy, such as heat, light or electricity...
-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptorThus, the two substrates
Substrate (biochemistry)
In biochemistry, a substrate is a molecule upon which an enzyme acts. Enzymes catalyze chemical reactions involving the substrate. In the case of a single substrate, the substrate binds with the enzyme active site, and an enzyme-substrate complex is formed. The substrate is transformed into one or...
of this enzyme are (S)-2-hydroxyglutarate
Alpha-Hydroxyglutaric acid
α-Hydroxyglutaric acid is an alpha hydroxy acid. In humans the compound is formed by a hydroxyacid-oxoacid transhydrogenase whereas in bacteria is formed by a 2-hydroxyglutarate synthase. The compound can be converted to α-ketoglutaric acid through the action of a 2-hydroxyglutarate dehydrogenase...
and acceptor
Electron acceptor
An electron acceptor is a chemical entity that accepts electrons transferred to it from another compound. It is an oxidizing agent that, by virtue of its accepting electrons, is itself reduced in the process....
, whereas its two products
Product (chemistry)
Product are formed during chemical reactions as reagents are consumed. Products have lower energy than the reagents and are produced during the reaction according to the second law of thermodynamics. The released energy comes from changes in chemical bonds between atoms in reagent molecules and...
are 2-oxoglutarate and reduced acceptor. However, enzymes with specificity to (R)-2-hydroxyglutarate dehydrogenase also exist in both mammals and plants even though they have not been given a unique EC number. This enzyme participates in butanoate metabolism.
Nomenclature
This enzyme belongs to the family of oxidoreductaseOxidoreductase
In biochemistry, an oxidoreductase is an enzyme that catalyzes the transfer of electrons from one molecule to another...
s, specifically those acting on the CH-OH group of donor with other acceptors. The systematic name of this enzyme class is (S)-2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:
- (S)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
- alpha-hydroxyglutarate dehydrogenase
- alpha-hydroxyglutarate dehydrogenase (NAD+ specific)
- alpha-hydroxyglutarate oxidoreductase
- alpha-ketoglutarate reductase
- hydroxyglutaric dehydrogenase
- L-alpha-hydroxyglutarate dehydrogenase
- L-alpha-hydroxyglutarate:NAD+ 2-oxidoreductase
Clinical significance
Deficiency in this enzyme in humans (D2HGDHD2HGDH
D-2-hydroxyglutarate dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the D2HGDH gene.-See also:* L2HGDH* 2-hydroxyglutarate synthase* 2-hydroxyglutarate dehydrogenase* Hydroxyacid-oxoacid transhydrogenase-Further reading:...
) or in the model plant Arabidopsis thaliana
Arabidopsis thaliana
Arabidopsis thaliana is a small flowering plant native to Europe, Asia, and northwestern Africa. A spring annual with a relatively short life cycle, arabidopsis is popular as a model organism in plant biology and genetics...
leads to massive accumulation of D-2-hydroxyglutarate
Alpha-Hydroxyglutaric acid
α-Hydroxyglutaric acid is an alpha hydroxy acid. In humans the compound is formed by a hydroxyacid-oxoacid transhydrogenase whereas in bacteria is formed by a 2-hydroxyglutarate synthase. The compound can be converted to α-ketoglutaric acid through the action of a 2-hydroxyglutarate dehydrogenase...
. In humans this results in the fatal neurometabolic disorder 2-Hydroxyglutaric aciduria whereas plants seem to be to a large extent unaffected by high cellular concentrations of this compound.
See also
- L2HGDHL2HGDHL-2-hydroxyglutarate dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the L2HGDH gene.-See also:* D2HGDH* 2-hydroxyglutarate synthase* 2-hydroxyglutarate dehydrogenase* Hydroxyacid-oxoacid transhydrogenase-Further reading:...
- 2-hydroxyglutarate synthase2-hydroxyglutarate synthaseIn enzymology, a 2-hydroxyglutarate synthase is an enzyme that catalyzes the chemical reactionThe 3 substrates of this enzyme are propanoyl-CoA, H2O, and glyoxylate, whereas its two products are 2-hydroxyglutarate and CoA....
- Alpha-Hydroxyglutaric acidAlpha-Hydroxyglutaric acidα-Hydroxyglutaric acid is an alpha hydroxy acid. In humans the compound is formed by a hydroxyacid-oxoacid transhydrogenase whereas in bacteria is formed by a 2-hydroxyglutarate synthase. The compound can be converted to α-ketoglutaric acid through the action of a 2-hydroxyglutarate dehydrogenase...
- 2-Hydroxyglutaric aciduria
- Hydroxyacid-oxoacid transhydrogenaseHydroxyacid-oxoacid transhydrogenaseIn enzymology, a hydroxyacid-oxoacid transhydrogenase is an enzyme that catalyzes the chemical reaction-3-hydroxybutanoate + 2-oxoglutarate \rightleftharpoons acetoacetate + -2-hydroxyglutarate...