α-hydroxy acid. Normally metabolized to 2-oxoglutarate (α-ketoglutarate) by L-2-hydroxyglutarate dehydrogenases, and mutations in this enzyme cause L-2-hydroxyglutaric aciduria, a neurometabolic disorder. Structurally similar to α-ketoglutarate and competitively inhibits α-ketoglutarate-dependent dioxygenases, including lysine demethylases and DNA hydroxylases.
Store at -20°C. Store under desiccating conditions. The product can be stored for up to 12 months.
Wherever possible, you should prepare and use solutions on the same day. However, if you need to make up stock solutions in advance, we recommend that you store the solution as aliquots in tightly sealed vials at -20°C. Generally, these will be useable for up to one month. Before use, and prior to opening the vial we recommend that you allow your product to equilibrate to room temperature for at least 1 hour.
da Rosa MS et al. In vivo intracerebral administration of L-2-hydroxyglutaric acid provokes oxidative stress and histopathological alterations in striatum and cerebellum of adolescent rats. Free Radic Biol Med83:201-13 (2015).
Read more (PubMed: 25701435) »
Xu W et al. Oncometabolite 2-hydroxyglutarate is a competitive inhibitor of a-ketoglutarate-dependent dioxygenases. Cancer Cell19:17-30 (2011).
Read more (PubMed: 21251613) »
Rzem R et al. A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria. Proc Natl Acad Sci U S A101:16849-54 (2004).
Read more (PubMed: 15548604) »