An internal standard for the quantification of ethylmalonic acid
Technical Support & Resources

Visit our FAQ

Contact Us

Toll Free Phone (USA and Canada Only): (888) 526-5351
Direct Phone: (734) 975-3888

Request Technical Support

Technical Support Request

To streamline the process attach the appropriate questionnaire to your inquiry.

Download IHC QuestionnaireDownload WB Questionnaire

View Our Privacy Statement for details on how we use and protect your data. In addition, this site is protected by hCaptcha and its Privacy Policy and Terms of Service apply.

Ethylmalonic Acid-d3

Item No. 43923

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
ethyl-2,2,2-d3-propanedioic acid
CAS Number
70907-93-6
Synonyms
  • EMA-d3
Molecular Formula
C5H5D3O4
Formula Weight
135.1
Purity
≥99% deuterated forms (d1-d3)
Formulation (Request formulation change)
A solid
Solubility(Learn about Variance in Solubility)
DMSO: Soluble: ≥10 mg/mlEthanol: Soluble: ≥10 mg/mlPBS pH 7.2: Sparingly soluble: 1-10 mg/ml
SMILES
[2H]C([2H])(CC(C(O)=O)C(O)=O)[2H]
InChi Code
InChI=1S/C5H8O4/c1-2-3(4(6)7)5(8)9/h3H,2H2,1H3,(H,6,7)(H,8,9)/i1D3
InChi Key
UKFXDFUAPNAMPJ-FIBGUPNXSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
Recommended Products

Certificates of Analysis & Batch Specific Data

Provide batch numbers separated by commas to download or request available product inserts, QC sheets, certificates of analysis, data packs, and GC-MS data.

Download most recent Certificate of Analysis for this product

    1 mg

    $32

    Add

    5 mg

    $72

    Add

    10 mg

    $128

    Add

    25 mg

    $280

    Add
    Product Description

    Ethylmalonic acid-d3 is intended for use as an internal standard for the quantification of ethylmalonic acid by GC- or LC-MS. 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).