04725
3-Hydroxyglutaric acid
analytical standard
Synonym(s):
β-Hydroxyglutaric acid, 3-Hydroxypentanedioic acid
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About This Item
Empirical Formula (Hill Notation):
C5H8O5
CAS Number:
Molecular Weight:
148.11
Beilstein:
1705476
MDL number:
UNSPSC Code:
12352106
PubChem Substance ID:
NACRES:
NA.24
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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.
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Storage Class Code
11 - Combustible Solids
WGK
WGK 3
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