(2S)-Octyl-a-hydroxyglutarate

a-Hydroxyglutaric acid (2-HG; Item No. 16374) is normally metabolized to 2-oxoglutarate by D- and L-2-hydroxyglutarate dehydrogenases. Mutations in these enzymes cause 2-hydroxyglutaric aciduria, a neurometabolic disorder.{26770,26771,26772} Recent studies have found that mutations in isocitrate dehydrogenase 1 (IDH1) and IDH2, typically associated with certain cancers, can cause these enzymes to convert isocitrate to 2-HG, rather than a-ketoglutarate.{26773,26775} 2-HG is structurally similar to a-ketoglutarate and competitively inhibits a-ketoglutarate-dependent dioxygenases, including lysine demethylases and DNA hydroxylases.{26775,26774,26769} (2S)-Octyl-a-hydroxyglutarate is a cell-permeable derivative of the L-isomer of 2-HG.