04725

3-Hydroxyglutaric acid

analytical standard

• Synonym(s): β-Hydroxyglutaric acid, 3-Hydroxypentanedioic acid
• Empirical Formula (Hill Notation): C₅H₈O₅
• CAS Number: 638-18-6
• Molecular Weight: 148.11
• Beilstein: 1705476
• MDL number: MFCD06208235
• UNSPSC Code: 12352106
• PubChem Substance ID: 329747978
• NACRES: NA.24

Properties

• grade: analytical standard
• Quality Level: 100
• Assay: ≥95.0% (GC)
• shelf life: limited shelf life, expiry date on the label
• application(s): clinical testing
• format: neat
• storage temp.: 2-8°C
• InChI: 1S/C5H8O5/c6-3(1-4(7)8)2-5(9)10/h3,6H,1-2H2,(H,7,8)(H,9,10)
• InChI key: ZQHYXNSQOIDNTL-UHFFFAOYSA-N

Description

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.

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Safety Information

• Storage Class Code: 11 - Combustible Solids
• WGK: WGK 3