| Size | Price | Stock | Qty |
|---|---|---|---|
| 50mg |
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| 100mg |
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| Other Sizes |
| Targets |
This compound is an endogenous metabolite that serves as a precursor of pantothenic acid in E. coli. The unlabeled form can increase levels of other branched-chain alpha-keto acids via transamination pathways. It is also a substrate for branched-chain alpha-keto acid dehydrogenase (BCKDH).
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| ln Vitro |
No direct in vitro pharmacological activity is reported; the labeled compound is used as a tracer. Unlabeled 3-methyl-2-oxobutanoic acid at 1-10 mM can act as a substrate for BCKDH in isolated enzyme assays and can be decarboxylated to isobutyryl-CoA.
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| ln Vivo |
In vivo, unlabeled 3-methyl-2-oxobutanoic acid (alpha-ketoisovaleric acid) has been shown to induce convulsions in rats at high doses via GABAergic and glutamatergic systems. It is also a key metabolite in maple syrup urine disease (MSUD).
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| Enzyme Assay |
A non-cell assay is performed with purified BCKDH complex or tissue homogenates. The 13C2-labeled compound is incubated at 37degC for 30-60 minutes in buffer containing CoA, NAD+, and thiamine pyrophosphate. Production of labeled CO2 or isobutyryl-CoA is measured by GC-MS or LC-MS.
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| Cell Assay |
For cell studies, the 13C2-labeled compound is added to culture medium of hepatocytes or E. coli at 0.1-1 mM. After 1-6 hours, cells are lysed and the incorporation of 13C into pantothenic acid or TCA cycle intermediates is analyzed by LC-MS.
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| Animal Protocol |
The compound is not intended for animal studies as a therapeutic. However, unlabeled alpha-ketoisovalerate is administered to rats by intraperitoneal injection (100-300 mg/kg) to model MSUD or study convulsions. Blood and brain metabolites are analyzed by GC-MS.
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| ADME/Pharmacokinetics |
The ADME properties are identical to unlabeled compound: it is a small organic acid rapidly absorbed from the gut, transported via monocarboxylate transporters, and metabolized by BCKDH in liver and muscle. It has a short plasma half-life of 30-60 minutes.
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| Toxicity/Toxicokinetics |
Unlabeled 3-methyl-2-oxobutanoic acid has low acute toxicity, but high doses (≥200 mg/kg in rats) can cause convulsions and neurotoxicity via GABA antagonism. At tracer doses, the labeled compound is non-toxic and safe for research use.
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| References |
[1]. Russak EM, et al. Impact of Deuterium Substitution on the Pharmacokinetics of Pharmaceuticals. Ann Pharmacother. 2019 Feb;53(2):211-216.
[2]. MAAS WK, et al. alpha-Ketoisovaleric acid, a precursor of pantothenic acid in Escherichia coli. J Bacteriol. 1953 Apr;65(4):388-93. [3]. Schauder P, et al. Oral administration of alpha-ketoisovaleric acid or valine in humans: blood kinetics and biochemical effects. J Lab Clin Med. 1984 Apr103(4):597-605. [4]. Coitinho AS, et al. Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats. Brain Res. 2001 Mar 9894(1):68-73. |
| Additional Infomation |
This labeled compound is used as an internal standard for LC-MS/MS quantification of 3-methyl-2-oxobutanoic acid in metabolomics and clinical diagnostics (e.g., MSUD). It is also used in tracer studies for branched-chain amino acid metabolism and pantothenate biosynthesis. Store at -20degC as a solid.
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| Molecular Formula |
C5H7NAO3
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|---|---|
| Molecular Weight |
140.082362413406
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| Exact Mass |
140.035
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| CAS # |
634908-42-2
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| Related CAS # |
Sodium 3-methyl-2-oxobutanoate;3715-29-5
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| PubChem CID |
23697315
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| Appearance |
White to off-white solid powder
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| Hydrogen Bond Donor Count |
0
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| Hydrogen Bond Acceptor Count |
3
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| Rotatable Bond Count |
2
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| Heavy Atom Count |
9
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| Complexity |
119
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| Defined Atom Stereocenter Count |
0
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| SMILES |
[Na+].O=C(C(=O)[O-])C([13CH3])[13CH3]
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| InChi Key |
WIQBZDCJCRFGKA-AWQJXPNKSA-M
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| InChi Code |
InChI=1S/C5H8O3.Na/c1-3(2)4(6)5(7)8;/h3H,1-2H3,(H,7,8);/q;+1/p-1/i1+1,2+1;
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| Chemical Name |
sodium;3-(113C)methyl-2-oxo(413C)butanoate
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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 | 7.1388 mL | 35.6939 mL | 71.3878 mL | |
| 5 mM | 1.4278 mL | 7.1388 mL | 14.2776 mL | |
| 10 mM | 0.7139 mL | 3.5694 mL | 7.1388 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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