3-Hydroxyglutaric Acid Analytical Standard., Each

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3-Hydroxyglutaric acid analytical standard.Synonym: β-hydroxyglutaric acid, 3-hydroxypentanedioic acid. Assay ≥95.0% (Gc). 3-Hydroxyglutaric acid is a glutaric acid derivative which is the byproduct of glutaric acidemia type i. Glutaric aciduria type i (glutaryl-coa dehydrogenase deficiency) is an inborn error of metabolism that usually manifests in infancy by an acute encephalopathic crisis and often results in permanent motor handicap. Studies indicate that 3-hydroxyglutaric acid can be used as biomarker for gcdh (glutaryl-coa dehydrogenase) deficiency. It is believed that the excretion of 3-hydroxyglutaric acid is increased during ketosis, which occurs during glutaryl-coa dehydrogenase deficiency. Studies on striatal cultures show that igf-1 and fgf-2 (bfgf) reduces 3-hydroxyglutaric acid toxicity in striatal neurons.