L-?-Hydroxyglutaric Acid

L-?-Hydroxyglutaric Acid

CAT N°: 21123
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L-?-Hydroxyglutaric acid (L-2-HG) is an ?-hydroxy acid. It is metabolized to 2-oxoglutarate (?-ketoglutarate) by L-2-hydroxyglutarate dehydrogenase, and mutations in this enzyme lead to 2-hydroxyglutaric aciduria, a neurometabolic disorder characterized by increased L-2-HG levels.{26770,26771} L-2-HG is structurally similar to ?-ketoglutarate and competitively inhibits ?-ketoglutarate-dependent dioxygenases, including several involved in histone lysine and DNA demethylation.{26769,26774}

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