A biomarker for GCDH deficiency
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3-Hydroxyglutaric Acid

Item No. 16334

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
3-hydroxy-pentanedioic acid
CAS Number
638-18-6
Synonyms
  • 2,4-Dideoxypentaric Acid
  • FA 5:1;O3
  • 3-HG
  • 3-Hydroxyglutarate
  • β-Hydroxyglutaric Acid
  • 3-Hydroxypentanedioic Acid
  • 3-OH-GA
Molecular Formula
C5H8O5
Formula Weight
148.1
Purity
≥95%
Formulation (Request formulation change)
A crystalline solid
Solubility(Learn about Variance in Solubility)
DMF: 30 mg/mlDMSO: 30 mg/mlEthanol: 30 mg/mlPBS (pH 7.2): 10 mg/ml
SMILES
OC(CC(O)CC(O)=O)=O
InChi Code
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
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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Certificates of Analysis & Batch Specific Data

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    1 mg

    $40

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    5 mg

    $129

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    10 mg

    $179

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    25 mg

    $393

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    Product Description

    3-Hydroxyglutaric acid is one of several metabolites produced when insufficient levels of GCDH are available.1,2 Urinary levels of 3-hydroxyglutaric acid are elevated during glutaric acidemia type 1, an autosomal recessive GCDH deficiency disorder that can lead to neurodegeneration if left untreated.1,2 3-Hydroxyglutaric acid is used as a biomarker of GCDH deficiency.1,2

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Fang, N., and Casida, J.E. Anticancer action of cubé insecticide: Correlation for rotenoid constituents between inhibition of NADH: Ubiquinone oxidoreductase and induced ornithine decarboxylase activities. Proc. Natl. Acad. Sci. USA 95(7), 3380-3384 (1998).

    2. Sauer, S.W., Okun, J.G., Schwab, M.A., et al. Bioenergetics in glutaryl-coenzyme a dehydrogenase deficiency. A role for glutaryl-coenzyme A. The Journal of Biological Chemisty 290(23), 21830-21836 (2005).