α-Hydroxyglutaric Acid (sodium salt)

α-Hydroxyglutaric Acid (sodium salt)

CAT N°: 16374
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From 74.00 62.90

?-Hydroxyglutaric acid (2-HG) is an ?-hydroxy acid. It is metabolized to 2-oxoglutarate by D- and L-2-hydroxyglutarate dehydrogenases, and mutations in these enzymes lead to 2-hydroxyglutaric aciduria, a neurometabolic disorder characterized by increased levels of L-2-hydroxyglutaric acid (Item No. 21123).{26770,26771,26772} 2-HG can also be metabolized from isocitrate by mutated forms of isocitrate dehydrogenase 1 (IDH1) and IDH2.{26773,26775} 2-HG is structurally similar to ?-ketoglutarate, the product of wild-type isocitrate dehydrogenases, and competitively inhibits ?-ketoglutarate-dependent dioxygenases, including histone lysine demethylases and DNA hydroxylases.{26775,26774,26769}

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