Synonyms: beta-Hydroxyglutaric acid; 3-Hydroxypentanedioic acid
Molecular Formula: C5H8O5
Molecular Weight: 148.11
Linear Structural Formula: C5H8O5
MDL Number: MFCD06208235
Purity: >=95.0% (GC)
Storage: 2-8C
Biochem Physiol Actions: 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.