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| D-2-HYDROXYPENTANEDIOIC ACID DISODIUM SALT Basic information |
| D-2-HYDROXYPENTANEDIOIC ACID DISODIUM SALT Chemical Properties |
| Melting point | >291°C (dec.) | | storage temp. | -20°C | | optical activity | [α]/D 8.5±1.5°, c = 1 in NaOH | | BRN | 5318041 | | InChIKey | DZHFTEDSQFPDPP-HWYNEVGZSA-L |
| WGK Germany | 3 | | HS Code | 29181990 |
| D-2-HYDROXYPENTANEDIOIC ACID DISODIUM SALT Usage And Synthesis |
| Description | D-α-Hydroxyglutaric Acid (D-2-HG) is an α-hydroxy acid that is over-produced in the human neurometabolic disease D-2-hydroxyglutaric aciduria (D-2-HGA). It is normally synthesized from 2-ketoglutarate (2-KG) by hydroxyacid-oxoacid transhydrogenase (HOT), although defects in HOT are not known to be associated with D-2-HGA. Instead, type I D-2-HGA involves mutations in D-2-hydroxyglutarate dehydrogenase, which converts D-2-HG back to 2-KG. Type II D-2-HGA results from gain-of-function mutations in isocitrate dehydrogenase 2, causing it to supplement HOT in converting 2-KG to D-2-HG. In bacteria, this α-hydroxy acid may be synthesized from oxalate and propionyl-coenzyme A by an α-hydroxyglutaric acid synthetase. | | Chemical Properties | Pale Orange Solid | | Uses | A potential inhibitor of glutamate carboxypeptidase. | | Biochem/physiol Actions | Biomarker for inborn errors of metabolism and cancer |
| D-2-HYDROXYPENTANEDIOIC ACID DISODIUM SALT Preparation Products And Raw materials |
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