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  • The base form of alpha-hydroxyglutaric acid, which is an intermediate in the synthesis of alpha-ketoglutaric acid. Alpha-hydroxyglutarate accumulates in the blood of patients with 2-hydroxyglutaric aciduria; an organic acid metabolism disorder, which is caused by the genetic mutation of one of the enzymes responsible for the conversion of 2-hydroxyglutaric acid to alpha-ketoglutaric acid.
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