α-Hydroxyglutaric acid (2-hydroxyglutaric acid) is an alpha hydroxy acid

Alpha hydroxy acid

α-Hydroxy acids, or alpha hydroxy acids , are a class of chemical compounds that consist of a carboxylic acid substituted with a hydroxyl group on the adjacent carbon. They may be either naturally occurring or synthetic. AHAs are well-known for their use in the cosmetics industry...

. In humans the compound is formed by a hydroxyacid-oxoacid transhydrogenase

Hydroxyacid-oxoacid transhydrogenase

In enzymology, a hydroxyacid-oxoacid transhydrogenase is an enzyme that catalyzes the chemical reaction-3-hydroxybutanoate + 2-oxoglutarate \rightleftharpoons acetoacetate + -2-hydroxyglutarate...

 whereas in bacteria is formed by a 2-hydroxyglutarate synthase

2-hydroxyglutarate synthase

In 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....

. The compound can be converted to α-ketoglutaric acid through the action of a 2-hydroxyglutarate dehydrogenase

2-hydroxyglutarate dehydrogenase

In enzymology, a 2-hydroxyglutarate dehydrogenase is an enzyme that catalyzes the chemical reaction-2-hydroxyglutarate + acceptor \rightleftharpoons 2-oxoglutarate + reduced acceptor...

 which, in humans, are two enzymes called D2HGDH

D2HGDH

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:...

 and L2HGDH

L2HGDH

L-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:...

. Deficiency in either of these two enzymes lead to a disease known as 2-hydroxyglutaric aciduria.