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04725

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3-Hydroxyglutaric acid

analytical standard

Synonym(s):
β-Hydroxyglutaric acid, 3-Hydroxypentanedioic acid
Empirical Formula (Hill Notation):
C5H8O5
CAS Number:
Molecular Weight:
148.11
Beilstein:
1705476
MDL number:
PubChem Substance ID:
NACRES:
NA.24

grade

analytical standard

Quality Level

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

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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.

Storage Class Code

13 - Non Combustible Solids

WGK

WGK 3

Certificate of Analysis

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Certificate of Origin

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