An internal standard for the quantification of malonic acid
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Malonic Acid-d4

Item No. 45689

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
acid-1,3-d2, propanedioic-2,2-d2
CAS Number
813-56-9
Synonyms
  • C3:0-d4
  • FA 3:1;O2-d4
  • Propanedioic Acid-d4
Molecular Formula
C3D4O4
Formula Weight
108.1
Purity
≥98%
Formulation (Request formulation change)
A solid
SMILES
[2H]OC(C([2H])([2H])C(O[2H])=O)=O
InChi Code
InChI=1S/C3H4O4/c4-2(5)1-3(6)7/h1H2,(H,4,5)(H,6,7)/i1D2/hD2
InChi Key
OFOBLEOULBTSOW-BGOGGDMHSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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    5 g

    $45

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    10 g

    $81

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    Product Description

    Malonic acid-d4 is intended for use as an internal standard for the quantification of malonic acid by GC- or LC-MS. Malonic acid is a dicarboxylic acid. At physiological pH, malonic acid is deprotonated to malonate, which competitively inhibits mitochondrial complex II, also known as succinate dehydrogenase, or is converted into malonyl-coenzyme A (malonyl-CoA; Item No. 16455) for use in fatty acid biosynthesis.1,2 Malonic and methylmalonic acid levels are elevated in combined malonic and methylmalonic aciduria, a disorder characterized by deficiency in malonyl-CoA decarboxylase (MCD) or acyl-CoA synthetase family member 3 (ACSF3).3

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Pardee, A.B., and Potter, V.R. Malonate inhibition of oxidations in the Krebs tricarboxylic acid cycle. The Journal of Biological Chemisty 178(1), 241-250 (1949).

    2. Bowman, C.E., and Wolfgang, M.J. Role of the malonyl-CoA synthetase ACSF3 in mitochondrial metabolism. Adv. Biol. Regul. 71, 34-40 (2019).

    3. de Sain-van der Velden, M.G., van der Ham, M., Jans, J.J., et al. A new approach for fast metabolic diagnostics in CMAMMA. JMID Rep. 30, 15-22 (2016).