A branched-chain organic acid
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3-Methylglutaconic Acid

Item No. 34055

Technical Information
Formal Name
3-methyl-2-pentenedioic acid
CAS Number
5746-90-7
Synonyms
  • 3-MGA
  • β-Methylglutaconic Acid
  • 3-MGC Acid
  • NSC 249232
  • FA 6:2;O2
Molecular Formula
C6H8O4
Formula Weight
Purity
≥95% (mixture of E/Z isomers)
A solid
DMF: 30 mg/mlDMSO: 30 mg/mlEthanol: 30 mg/mlPBS (pH 7.2): 10 mg/ml
λmax
213 nm
SMILES
O=C(C=C(CC(O)=O)C)O
InChi Code
InChI=1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)
InChi Key
WKRBKYFIJPGYQC-UHFFFAOYSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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    Product Description

    3-Methylglutaconic acid (3-MGA) is a branched-chain organic acid and product of leucine catabolism.1 3-MGA accumulates in 3-methylglutaconic aciduria, which is caused by an inborn error of leucine metabolism characterized by 3-MGA-CoA hydratase deficiency, leading to delayed development, hypoglycemia, metabolic acidosis, and neurological impairments.2,1

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Wortmann, S.B., Kluijtmans, L.A., Engelke, U.F.H., et alThe 3-methylglutaconic acidurias: What’s new? J. Inherit. Metab. Dis. 35(1), 13-22 (2012).

    2. Leipnitz, G., Seminotti, B., Amaral, A.U., et alInduction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats. Life Sci. 82(11-12), 652-662 (2008).