α-Hydroxyglutaric acid is normally metabolized to 2-oxoglutarate by D- and L-2-hydroxyglutarate dehydrogenases.
Cas No. | 2097068-39-6 |
Canonical SMILES | OC(CCC(O)C(OCCCCCCCC)=O)=O |
分子式 | C13H24O5 |
分子量 | 260.3 |
溶解度 | DMF: 10 mg/ml,DMSO: 10 mg/ml,Ethanol: 20 mg/ml,PBS (pH 7.2): 5 mg/ml |
储存条件 | Store at -20°C |
General tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while. |
Shipping Condition | Evaluation sample solution : ship with blue ice All other available size: ship with RT , or blue ice upon request |
产品描述 | 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. |