(2S)-2-hydroxypentanedioic acid

• CAT Number: M054944
• CAS Number: 13095-48-2
• Molecular Formula: C5H8O5
• Molecular Weight: 148.114
• Purity: ≥95%
• Synonyms: 2(S)-HG;L-2-Hydroxyglutaric Acid;L-α-Hydroxyglutaric Acid
• Target: Metabolic Enzyme/Protease
• Storage: -20°C
• IUPAC Name: (2S)-2-hydroxypentanedioic acid
• InChI: InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)/t3-/m0/s1
• InChIKey: HWXBTNAVRSUOJR-VKHMYHEASA-N
• SMILES: C(CC(=O)O)C(C(=O)O)O

(2S)-Hydroxyglutaric acid is a natural α-hydroxy acid that is normally metabolized to 2-oxoglutarate (α-ketoglutarate) by L-2-hydroxyglutarate dehydrogenase (L2HGDH). Mutations in L2HGDH result in L-2-hydroxyglutaric aciduria, an autosomal recessive disorder resulting in neurological impairment at a young age. Mutations in isocitrate dehydrogenase 1 (IDH1) and IDH2, typically associated with certain cancers, can cause these enzymes to convert isocitrate to 2-hydroxyglutaric acid, rather than 2-oxoglutarate. (2S)-Hydroxyglutaric acid inhibits 2-oxoglutarate-dependent dioxygenases, including lysine demethylases and DNA hydroxylases.