Chemistry:Alpha-Hydroxyglutaric acid
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| Preferred IUPAC name
2-Hydroxypentanedioic acid | |
| Other names
2-Hydroxyglutaric acid
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3D model (JSmol)
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| MeSH | Alpha-hydroxyglutarate |
PubChem CID
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| Properties | |
| C5H8O5 | |
| Molar mass | 148.114 g·mol−1 |
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa). | |
 verify (what is   ?)
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| Infobox references | |
α-Hydroxyglutaric acid (2-hydroxyglutaric acid) is an alpha hydroxy acid form of glutaric acid.
In biology
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.
D-2-hydroxyglutarate
Mutations in isocitrate dehydrogenase (IDH1 and IDH2), which frequently occur in glioma and AML,[1][2][3] produce D-2-hydroxyglutarate from alpha-ketoglutarate.[4] D-2-hydroxyglutarate accumulates to very high concentrations which inhibits the function of enzymes that are dependent on alpha-ketoglutarate, including histone lysine demethylases.[5]Cite error: Closing </ref> missing for <ref> tag
References
- ↑ "Monoclonal antibody specific for IDH1 R132H mutation". Acta Neuropathol. 118 (5): 599–601. November 2009. doi:10.1007/s00401-009-0595-z. PMID 19798509.
- ↑ "The common feature of leukemia-associated IDH1 and IDH2 mutations is a neomorphic enzyme activity converting alpha-ketoglutarate to 2-hydroxyglutarate". Cancer Cell 17 (3): 225–34. March 2010. doi:10.1016/j.ccr.2010.01.020. PMID 20171147.
- ↑ "WT1 recruits TET2 to regulate its target gene expression and suppress leukemia cell proliferation". Molecular Cell 57 (4): 662–73. Feb 2015. doi:10.1016/j.molcel.2014.12.023. PMID 25601757.
- ↑ "Cancer-associated IDH1 mutations produce 2-hydroxyglutarate". Nature 465 (7300): 966. Jun 2010. doi:10.1038/nature09132. PMID 20559394.
- ↑ "The oncometabolite 2-hydroxyglutarate inhibits histone lysine demethylases". EMBO Rep 12 (5): 463–9. May 2011. doi:10.1038/embor.2011.43. PMID 21460794.
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