Chemical Properties
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Uses
3-Hydroxyglutaric Acid (cas# 638-18-6) is a compound useful in organic synthesis.
Definition
ChEBI: 3-hydroxyglutaric acid is a 3 hydroxy carboxylic acid that is glutaric acid which is substituted by a hydroxy group at position 3. It is a diagnostic marker for glutaric aciduria type I. It has a role as a human urinary metabolite and a human blood serum metabolite. It is a 3-hydroxy carboxylic acid and an alpha,omega-dicarboxylic acid. It is functionally related to a glutaric acid. It is a conjugate acid of a 3-hydroxyglutarate(2-).
Biological Activity
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 th at usually manifests in infancy by an acute encephalopathic crisis and often results in permanent motor handicap. Studies indicate th at 3-hydroxyglutaric acid can be used as biomarker for GCDH (glutaryl-CoA dehydrogenase) deficiency. It is believed th at the excretion of 3-hydroxyglutaric acid is increased during ketosis, which occurs during glutaryl-CoA dehydrogenase deficiency. Studies on striatal cultures show th at IGF-1 and FGF-2 (bFGF) reduces 3-hydroxyglutaric acid toxicity in striatal neurons.
