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2-Hydroxyglutaric Acidurias The 2-hydroxyglutaric acidurias represents a family of related metabolic disorders that are due to the accumulation of one or the other, or both, of the stereoisomers of 2-hydroxyglutarate (2-HG), namely D-2-hydroxyglutaric acid and L-2-hydroxyglutaric acid in the blood, cerebrospinal fluid (CSF), and the urine
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