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About This Item
Empirical Formula (Hill Notation):
C5H8O5
CAS Number:
Molecular Weight:
148.11
NACRES:
NA.24
PubChem Substance ID:
UNSPSC Code:
12352106
MDL number:
Beilstein/REAXYS Number:
1705476
grade
analytical standard
Quality Segment
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
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.
Other Notes
Find a digital Reference Material for this product available on our online platform ChemisTwin® for NMR. You can use this digital equivalent on ChemisTwin® for your sample identity confirmation and compound quantification (with digital external standard). An NMR spectrum of this substance can be viewed and an online comparison against your sample can be performed with a few mouseclicks. Learn more here and start your free trial.
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Storage Class
11 - Combustible Solids
wgk
WGK 3
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