An internal standard for the quantification of methylmalonic acid
Related Products
Unlabeled Version(s)
14885Methylmalonic Acid
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Methylmalonic Acid-d3

Item No. 35232

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
2-methyl-d3-propanedioic acid
CAS Number
42522-59-8
Synonyms
  • Isosuccinic Acid-d3
  • Methylmalonate-d3
  • MMA-d3
Molecular Formula
C4H3D3O4
Formula Weight
121.1
Purity
≥99% deuterated forms (d1-d3)
Formulation
A solid
Solubility(Learn about Variance in Solubility)
DMSO: slightlyMethanol: slightly
SMILES
OC(C(C([2H])([2H])[2H])C(O)=O)=O
InChi Code
InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)/i1D3
InChi Key
ZIYVHBGGAOATLY-FIBGUPNXSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
Certificates of Analysis & Batch Specific Data

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    5 mg

    $86

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    10 mg

    $154

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    25 mg

    $343

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    50 mg

    $598

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    Product Description

    Methylmalonic acid-d3 is intended for use as an internal standard for the quantification of methylmalonic acid (Item No. 14885) by GC- or LC-MS. Methylmalonic acid is a dicarboxylic acid and byproduct of propionate catabolism.1 It impairs mitochondrial respiration, increases glucose consumption, and downregulates the transcription of the mature neuronal markers neuron-specific enolase (ENO2) and synaptophysin (SYP) in neuronally differentiated SH-SY5Y cells. Intrastriatal administration of methylmalonic acid (100 mg/ml) induces neuronal loss in rats.2 Methylmalonic acid accumulates in the tissues and body fluids of patients with methylmalonic acidemia, an inborn error of metabolism characterized by a deficiency in the activity of L-methylmaolnyl-CoA mutase or its cofactor 5-deoxyadenosylcobalamin and leads to seizures, intellectual disabilities, psychomotor abnormalities, and coma.1

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. da Costa, R.T., Dos Santos, M.B., Silva, I.C.S., et al. Methylmalonic acid compromises respiration and reduces the expression of differentiation markers of SH-SY5Y human neuroblastoma cells. ACS Chem. Neurosci. 12(14), 2608-2618 (2021).

    2. Narasimhan, P., Sklar, R., Murrell, M., et al. Methylmalonyl-CoA mutase induction by cerebral ischemia and neurotoxicity of the mitochondrial toxin methylmalonic acid. J. Neurosci. 16(22), 7336-7346 (1996).