A dicarboxylic acid and metabolite of L-isoleucine and L-alloisoleucine
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Isomer(s)
36430Methylsuccinic Acid
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Ethylmalonic Acid

Item No. 44091

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
2-ethyl-propanedioic acid
CAS Number
601-75-2
Molecular Formula
C5H8O4
Formula Weight
132.1
Purity
≥98%
Formulation (Request formulation change)
A solid
Solubility(Learn about Variance in Solubility)
DMSO: Slightly soluble: 0.1-1 mg/mlEthanol: Sparingly soluble: 1-10 mg/mlPBS (pH 7.2): Slightly soluble: 0.1-1 mg/ml
SMILES
O=C(C(CC)C(O)=O)O
InChi Code
InChI=1S/C5H8O4/c1-2-3(4(6)7)5(8)9/h3H,2H2,1H3,(H,6,7)(H,8,9)
InChi Key
UKFXDFUAPNAMPJ-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 g

    $28

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    5 g

    $70

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    10 g

    $125

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    25 g

    $275

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    Product Description

    Ethylmalonic acid is a dicarboxylic acid and metabolite of the essential amino acid L-isoleucine and branched-chain amino acid L-alloisoleucine (Item No. 34904).1 It is formed from L-isoleucine and L-alloisoleucine via an R-2-oxo-3-methylvaleric acid intermediate. Urinary levels of ethylmalonic acid are increased in patients with ethylmalonic encephalopathy, an inborn error of metabolism characterized by developmental delay, hypotonia, vascular instability, petechiae, acrocyanosis, chronic diarrhea, and lactic acidemia. Urinary levels of ethylmalonic acid are also increased in patients with short-chain acyl-CoA dehydrogenase deficiency.2

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Nowaczyk, M.J.M., Lehotay, D.C., Platt, B.-A., et al. Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism 47(7), 836-839 (1998).

    2. Gallant, N.M., Leydiker, K., Tang, H., et al. Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California. Mol. Genet. Metab. 106(1), 55-61 (2012).