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2-hydroxyglutarate

α-Hydroxyglutaric acid
Alpha-hydroxyglutaric acid.png
Names
IUPAC name
2-Hydroxypentanedioic acid
Other names
2-Hydroxyglutaric acid
Identifiers
3D model (Jmol)
ChEBI
ChemSpider
KEGG
MeSH Alpha-hydroxyglutarate
PubChem CID
Properties
C5H8O5
Molar mass 148.11 g·mol−1
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
N  (what is YesYN ?)
Infobox references

α-Hydroxyglutaric acid (2-hydroxyglutaric acid) is an alpha hydroxy acid form of glutaric 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 which, in humans, are two enzymes called D2HGDH and L2HGDH. Deficiency in either of these two enzymes lead to a disease known as 2-hydroxyglutaric aciduria.

Mutations in isocitrate dehydrogenase (IDH1 and IDH2), which frequently occur in glioma and AML, produce (D)-2-hydroxyglutarate from alpha-ketoglutarate. (D)-2-hydroxyglutarate accumulates to very high concentrations which inhibits the function of enzymes that are dependent on alpha-ketoglutarate. This leads to a hypermethylated state of DNA and histones, which results in different gene expression that can activate oncogenes and inactivate tumor-suppressor genes.



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