[{"from": "human", "value": "Provide a description of this molecule. [C][Branch1][#Branch1][C][C][=Branch1][C][=O][O][C@H1][Branch1][=Branch1][C][=Branch1][C][=O][O][O]"}, {"from": "gpt", "value": "The molecule is an alpha hydroxy acid. In humans the compound is formed by a hydroxyacid-oxoacid transhydrogenase whereas in bacteria is formed by a 2-hydroxyglutarate synthase. D-2-Hydroxyglutarate is also formed via the normal activity of hydroxyacid-oxoacid transhydrogenase during conversion of 4-hydroxybutyrate to succinate semialdehyde. The compound can be converted to \u03b1-ketoglutaric acid through the action of a 2-hydroxyglutarate dehydrogenase. In humans, there are two such enzymes called D2HGDH and L2HGDH. Tissue accumulation of high amounts of D-2-hydroxyglutaric acid is the biochemical hallmark of the inherited neurometabolic disorder D-2-hydroxyglutaric aciduria. Both the D and the L stereoisomers of hydroxyglutaric acid (EC 1.1.99.2) are found in body fluids. Accumulation of L-2-hydroxyglutaric acid has been reported in multiple patients who have a clinical phenotype of progressive neurodegeneration with extrapyramidal and cerebellar signs, seizures, and spongiform changes in the white matter. Patients who have excess accumulation of D-2-hydroxyglutaric acid in the urine exhibit a variable phenotype but included mental retardation, macrocephaly with cerebral atrophy, hypotonia, seizures, and involuntary movements. D-2-hydroxyglutarate mediates its neurotoxicity through activation of N-methyl-D-aspartate receptors. D-2-hydroxyglutarate is structurally similar to the excitatory amino acid glutamate and stimulates neurodegeneration by mechanisms well-known for glutamate, NMDA or mitochondrial toxins (A15198)"}]