α-Hydroxyglutaric acid is normally metabolized to 2-oxoglutarate by D- and L-2-hydroxyglutarate dehydrogenases.
CAS：2097068-39-6
分子式：C13H24O5
分子量：260.3
纯度：98%
存储：Store at -20°C

Background:

α-Hydroxyglutaric acid is normally metabolized to 2-oxoglutarate by D- and L-2-hydroxyglutarate dehydrogenases. Mutations in these enzymes cause 2-hydroxyglutaric aciduria, a neurometabolic disorder. 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 α-ketoglutarate. 2-HG is structurally similar to α-ketoglutarate and competitively inhibits α-ketoglutarate-dependent dioxygenases, including lysine demethylases and DNA hydroxylases. Octyl-α-hydroxyglutarate is a cell-permeable derivative of 2-HG.