enzymology Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. A ...

, an L-2-hydroxyglutarate dehydrogenase () is an

enzyme Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. A ...

that catalyzes the

chemical reaction A chemical reaction is a process that leads to the IUPAC nomenclature for organic transformations, chemical transformation of one set of chemical substances to another. Classically, chemical reactions encompass changes that only involve the pos ...

:(S)-2-hydroxyglutarate + acceptor

\rightleftharpoons

2-oxoglutarate + reduced acceptor Thus, the two substrates of this enzyme are (S)-2-hydroxyglutarate and acceptor, whereas its two

products Product may refer to: Business * Product (business), an item that serves as a solution to a specific consumer problem. * Product (project management), a deliverable or set of deliverables that contribute to a business solution Mathematics * Produ ...

are 2-oxoglutarate and

reduced 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. Electron acceptors are sometimes mista ...

. Enzymes which preferentially catalyze the conversion of the (R) stereoisomer of 2-oxoglutarate also exist in both mammals and plants and are named

D-2-hydroxyglutarate dehydrogenase In enzymology, a D-2-hydroxyglutarate dehydrogenase () is an enzyme that catalyzes the chemical reaction :(R)-2-hydroxyglutarate + acceptor \rightleftharpoons 2-oxoglutarate + reduced acceptor Thus, the two substrates of this enzyme are (R)-2- ...

. L-2-hydroxyglutarate is produced by promiscuous action of

malate dehydrogenase Malate dehydrogenase () (MDH) is an enzyme that reversibly catalyzes the oxidation of malate to oxaloacetate using the reduction of NAD+ to NADH. This reaction is part of many metabolic pathways, including the citric acid cycle. Other malate ...

on 2-oxoglutarate; L-2-hydroxyglutarate dehydrogenase is an example of a metabolite repair enzyme that oxidizes L-2-hydroxyglutarate back to 2-oxoglutarate.

Nomenclature

This enzyme belongs to the family of

oxidoreductase In biochemistry, an oxidoreductase is an enzyme that catalyzes the transfer of electrons from one molecule, the reductant, also called the electron donor, to another, the oxidant, also called the electron acceptor. This group of enzymes usually ut ...

s, specifically those acting on the CH-OH group of donor with other acceptors. The

systematic name A systematic name is a name given in a systematic way to one unique group, organism, object or chemical substance, out of a specific population or collection. Systematic names are usually part of a nomenclature. A semisystematic name or semitrivial ...

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 (

L2HGDH L-2-hydroxyglutarate dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the ''L2HGDH'' gene, also known as C14orf160, on chromosome 14. Function This gene encodes L-2-hydroxyglutarate dehydrogenase, a flavin adenine dinucle ...

) or in the model plant ''

Arabidopsis thaliana ''Arabidopsis thaliana'', the thale cress, mouse-ear cress or arabidopsis, is a small flowering plant native to Eurasia and Africa. ''A. thaliana'' is considered a weed; it is found along the shoulders of roads and in disturbed land. A winter a ...

'' (At3g56840) leads to accumulation of L-2-hydroxyglutarate. In humans this results in the fatal neurometabolic disorder

2-Hydroxyglutaric aciduria 2-hydroxyglutaric aciduria is a rare neurometabolic disorder characterized by the significantly elevated levels of hydroxyglutaric acid in one's urine. It is either autosomal recessive or autosomal dominant. Presentation The signs/symptoms of thi ...

whereas plants seem to be unaffected by elevated cellular concentrations of this compound

Nomenclature

Clinical significance

See also

References

Further reading