| Size | Price | Stock | Qty |
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| 1mg |
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| 5mg |
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| Other Sizes |
| Targets |
Ethylmalonic acid is a metabolite in fatty acid metabolism. Elevated levels are associated with malonyl-CoA decarboxylase deficiency and ethylmalonic encephalopathy. The deuterated form is used as an internal standard for quantification, not as a pharmacological agent.
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| ln Vitro |
The labeled compound has no in vitro pharmacological activity. Unlabeled ethylmalonic acid (1-10 mM) can inhibit mitochondrial fatty acid oxidation and cause cellular toxicity in hepatocytes. It is also a substrate for malonyl-CoA decarboxylase (MCD).
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| ln Vivo |
No in vivo activity is reported for the labeled compound. Unlabeled ethylmalonic acid is a toxic metabolite; elevated levels in patients cause neurological symptoms, metabolic acidosis, and developmental delay. In animal models, ethylmalonate infusion induces neurotoxicity.
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| Enzyme Assay |
A non-cell assay for MCD activity uses purified enzyme and ethylmalonyl-CoA as substrate. For analytical use, Ethylmalonic acid-d5 is spiked into calibration standards and samples at a fixed concentration (e.g., 10-100 ng/mL) before LC-MS/MS analysis. Protein precipitation or SPE is followed by MRM detection.
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| Cell Assay |
Cell-based assays are not applicable for this compound as a pharmacological agent. In analytical contexts, the compound may be added to culture medium as an internal standard during method validation for studies of fatty acid metabolism in hepatocyte cell lines.
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| Animal Protocol |
The deuterated compound is not administered to animals. Instead, it is added post-collection to plasma, urine, or tissue homogenates from animal models of fatty acid oxidation disorders (e.g., MCD knockout mice) as an internal standard for LC-MS/MS quantification of endogenous ethylmalonic acid.
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| ADME/Pharmacokinetics |
Ethylmalonic acid-d5 has identical ADME to unlabeled ethylmalonic acid: it is a small polar dicarboxylic acid (MW 137.15) that is renally cleared and has a short half-life (hours). As a deuterated internal standard, it co-elutes with the analyte but is distinguished by mass shift (+5 Da).
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| Toxicity/Toxicokinetics |
Unlabeled ethylmalonic acid is potentially toxic. Elevated levels cause metabolic acidosis, neurological damage, and developmental delay. The deuterated analog is safe at analytical concentrations (ng/mL range) and is handled under standard laboratory safety protocols.
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| References |
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| Additional Infomation |
Ethylmalonic acid-d5 is used as an internal standard for the diagnosis of malonyl-CoA decarboxylase deficiency, ethylmalonic encephalopathy, and other fatty acid oxidation disorders by LC-MS/MS. It is a critical reagent in newborn screening and clinical diagnostics. Purity ≥98 atom % D, store at -20degC.
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| Molecular Formula |
C5H3D5O4
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|---|---|
| Molecular Weight |
137.15
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| Exact Mass |
137.074
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| CAS # |
66311-22-6
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| Related CAS # |
Ethylmalonic acid;601-75-2
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| PubChem CID |
90474566
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| Appearance |
White to off-white solid powder
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| LogP |
0.181
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| Hydrogen Bond Donor Count |
2
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| Hydrogen Bond Acceptor Count |
4
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| Rotatable Bond Count |
3
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| Heavy Atom Count |
9
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| Complexity |
115
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| Defined Atom Stereocenter Count |
0
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| SMILES |
[2H]C([2H])([2H])C([2H])([2H])C(C(=O)O)C(=O)O
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| InChi Key |
UKFXDFUAPNAMPJ-ZBJDZAJPSA-N
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| InChi Code |
InChI=1S/C5H8O4/c1-2-3(4(6)7)5(8)9/h3H,2H2,1H3,(H,6,7)(H,8,9)/i1D3,2D2
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| Chemical Name |
2-(1,1,2,2,2-pentadeuterioethyl)propanedioic acid
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| HS Tariff Code |
2934.99.9001
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| Storage |
Powder -20°C 3 years 4°C 2 years In solvent -80°C 6 months -20°C 1 month |
| Shipping Condition |
Room temperature (This product is stable at ambient temperature for a few days during ordinary shipping and time spent in Customs)
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| Solubility (In Vitro) |
May dissolve in DMSO (in most cases), if not, try other solvents such as H2O, Ethanol, or DMF with a minute amount of products to avoid loss of samples
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| Solubility (In Vivo) |
Note: Listed below are some common formulations that may be used to formulate products with low water solubility (e.g. < 1 mg/mL), you may test these formulations using a minute amount of products to avoid loss of samples.
Injection Formulations
Injection Formulation 1: DMSO : Tween 80： Saline = 10 : 5 : 85 (i.e. 100 μL DMSO stock solution → 50 μL Tween 80 → 850 μL Saline)(e.g. IP/IV/IM/SC) *Preparation of saline: Dissolve 0.9 g of sodium chloride in 100 mL ddH ₂ O to obtain a clear solution. Injection Formulation 2: DMSO : PEG300 ：Tween 80 : Saline = 10 : 40 : 5 : 45 (i.e. 100 μL DMSO → 400 μLPEG300 → 50 μL Tween 80 → 450 μL Saline) Injection Formulation 3: DMSO : Corn oil = 10 : 90 (i.e. 100 μL DMSO → 900 μL Corn oil) Example: Take the Injection Formulation 3 (DMSO : Corn oil = 10 : 90) as an example, if 1 mL of 2.5 mg/mL working solution is to be prepared, you can take 100 μL 25 mg/mL DMSO stock solution and add to 900 μL corn oil, mix well to obtain a clear or suspension solution (2.5 mg/mL, ready for use in animals). View More
Injection Formulation 4: DMSO : 20% SBE-β-CD in saline = 10 : 90 [i.e. 100 μL DMSO → 900 μL (20% SBE-β-CD in saline)] Oral Formulations
Oral Formulation 1: Suspend in 0.5% CMC Na (carboxymethylcellulose sodium) Oral Formulation 2: Suspend in 0.5% Carboxymethyl cellulose Example: Take the Oral Formulation 1 (Suspend in 0.5% CMC Na) as an example, if 100 mL of 2.5 mg/mL working solution is to be prepared, you can first prepare 0.5% CMC Na solution by measuring 0.5 g CMC Na and dissolve it in 100 mL ddH2O to obtain a clear solution; then add 250 mg of the product to 100 mL 0.5% CMC Na solution, to make the suspension solution (2.5 mg/mL, ready for use in animals). View More
Oral Formulation 3: Dissolved in PEG400 (Please use freshly prepared in vivo formulations for optimal results.) |
| Preparing Stock Solutions | 1 mg | 5 mg | 10 mg | |
| 1 mM | 7.2913 mL | 36.4564 mL | 72.9129 mL | |
| 5 mM | 1.4583 mL | 7.2913 mL | 14.5826 mL | |
| 10 mM | 0.7291 mL | 3.6456 mL | 7.2913 mL |
*Note: Please select an appropriate solvent for the preparation of stock solution based on your experiment needs. For most products, DMSO can be used for preparing stock solutions (e.g. 5 mM, 10 mM, or 20 mM concentration); some products with high aqueous solubility may be dissolved in water directly. Solubility information is available at the above Solubility Data section. Once the stock solution is prepared, aliquot it to routine usage volumes and store at -20°C or -80°C. Avoid repeated freeze and thaw cycles.
Calculation results
Working concentration： mg/mL；
Method for preparing DMSO stock solution： mg drug pre-dissolved in μL DMSO (stock solution concentration mg/mL). Please contact us first if the concentration exceeds the DMSO solubility of the batch of drug.
Method for preparing in vivo formulation:：Take μL DMSO stock solution, next add μL PEG300, mix and clarify, next addμL Tween 80, mix and clarify, next add μL ddH2O，mix and clarify.
(1) Please be sure that the solution is clear before the addition of next solvent. Dissolution methods like vortex, ultrasound or warming and heat may be used to aid dissolving.
(2) Be sure to add the solvent(s) in order.
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