| Size | Price | Stock | Qty |
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| 5mg |
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| 10mg |
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| Targets |
The primary "target" of 3-Methylcrotonylglycine is not a therapeutic target, but rather its role as a diagnostic biomarker. It is formed from the metabolism of leucine when the enzyme 3-methylcrotonyl-CoA carboxylase (3-MCC) is deficient. It is a metabolite that accumulates in the body and is excreted in the urine. Its detection and quantification in biological samples, such as urine and dried blood spots, are used as a diagnostic tool for inborn errors of metabolism. It does not have a known receptor or enzyme that it modulates.
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| ln Vitro |
In vitro, 3-Methylcrotonylglycine is used as an analytical reference standard. It is employed in mass spectrometry and chromatography to identify and quantify metabolites in biological samples. It has been shown to disrupt mitochondrial energy homeostasis and inhibit synaptic Na+, K+-ATPase activity in the brain of young rats, indicating potential biological effects, but its primary use is as a diagnostic marker. It does not have a defined therapeutic activity in vitro.
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| ln Vivo |
In vivo, 3-Methylcrotonylglycine is a biomarker that accumulates in the body in the context of metabolic disorders such as 3-methylcrotonylglycinuria and organic acidemias. It is found in the urine and blood of affected individuals. Its levels are used to diagnose and monitor these conditions. It may have toxic effects, as it has been shown to inhibit Na+, K+-ATPase activity in the brain, but its primary significance is as a diagnostic tool. It is not administered as a therapeutic agent.
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| Enzyme Assay |
Cell-free assays for 3-Methylcrotonylglycine are not applicable as it is a biomarker, not a drug with a defined target. However, its detection and quantification in biological samples are performed using analytical techniques such as liquid chromatography-tandem mass spectrometry (LC-MS/MS) or gas chromatography-mass spectrometry (GC-MS). A typical protocol involves extracting the compound from a biological matrix (e.g., urine, dried blood spots), derivatizing it to improve volatility or ionization, and then analyzing it using a mass spectrometer. The compound is identified and quantified based on its retention time and specific mass transitions.
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| Cell Assay |
In vitro cellular experiments with 3-Methylcrotonylglycine are not typical, as it is a biomarker. However, its effects on cells can be studied. For example, neuronal cells can be cultured and treated with the compound at various concentrations (e.g., 0.1-10 mM) to assess its effect on Na+, K+-ATPase activity. Cell viability and mitochondrial function can also be assessed. These experiments are useful for understanding the potential pathophysiology associated with its accumulation in metabolic disorders.
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| Animal Protocol |
In vivo animal experiments with 3-Methylcrotonylglycine are typically conducted to model or study the effects of its accumulation. A common protocol involves administering the compound to young rats via intraperitoneal injection. The effects on brain Na+, K+-ATPase activity and mitochondrial energy homeostasis are then assessed. These experiments help elucidate the potential toxicity and pathophysiology of 3-methylcrotonylglycinuria and related organic acidemias.
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| ADME/Pharmacokinetics |
3-Methylcrotonylglycine has a molecular weight of 157.17 g/mol and a molecular formula of C7H11NO3. It is a small, polar molecule that is soluble in water and organic solvents. It is stable as a powder and can be stored at room temperature. As an endogenous metabolite, its pharmacokinetics are determined by its production and clearance in the body. It is primarily excreted in the urine. It is used as an analytical standard and is not administered as a drug.
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| Toxicity/Toxicokinetics |
3-Methylcrotonylglycine is a metabolite and is considered a diagnostic biomarker. As an endogenous compound, its toxicity is primarily associated with its accumulation in metabolic disorders. In high concentrations, it can disrupt mitochondrial energy homeostasis and inhibit Na+, K+-ATPase, potentially contributing to the neurological symptoms seen in organic acidemias. It is not administered as a therapeutic agent, so its safety profile is not applicable in that context. It is an analytical standard for research use.
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| Additional Infomation |
3-Methylcrotonylglycine is an N-acylglycine, in which the acyl group is specifically defined as 3-methylbut-2-enoyl. It is a metabolite. Its function is related to 3-methylbut-2-enoic acid and glycine. It has been reported that 3-methylcrotonylglycine exists in Homo sapiens and Apis cerana, and relevant data are available.
3-Methylcrotonylglycine (3-MCG) is a definitive pathognomonic biomarker for 3-methylcrotonyl-CoA carboxylase (3-MCC) deficiency and a diagnostic marker for organic acidemias. It is an N-acylglycine metabolite formed from leucine catabolism. It is used as an analytical reference standard in clinical chemistry, metabolomics, and newborn screening programs. Its quantification in urine and dried blood spots via LC-MS/MS is essential for accurate diagnosis. It is a research compound and not a therapeutic agent. |
| Molecular Formula |
C7H11NO3
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|---|---|
| Molecular Weight |
157.16714
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| Exact Mass |
157.074
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| CAS # |
33008-07-0
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| Related CAS # |
3-Methylcrotonylglycine-d2;1276197-31-9
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| PubChem CID |
169485
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| Appearance |
White to off-white solid powder
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| Density |
1.138g/cm3
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| Boiling Point |
394.9ºC at 760mmHg
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| Flash Point |
192.6ºC
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| Index of Refraction |
1.535
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| LogP |
0.544
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| Hydrogen Bond Donor Count |
2
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| Hydrogen Bond Acceptor Count |
3
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| Rotatable Bond Count |
3
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| Heavy Atom Count |
11
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| Complexity |
192
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| Defined Atom Stereocenter Count |
0
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| InChi Key |
PFWQSHXPNKRLIV-UHFFFAOYSA-N
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| InChi Code |
InChI=1S/C7H11NO3/c1-5(2)3-6(9)8-4-7(10)11/h3H,4H2,1-2H3,(H,8,9)(H,10,11)
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| Chemical Name |
2-(3-methylbut-2-enoylamino)acetic 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 Note: Please store this product in a sealed and protected environment, avoid exposure to moisture. |
| 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) |
DMSO : ~250 mg/mL (~1590.63 mM)
H2O : ~12.5 mg/mL (~79.53 mM) |
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| Solubility (In Vivo) |
Solubility in Formulation 1: ≥ 6.25 mg/mL (39.77 mM) (saturation unknown) in 10% DMSO + 90% (20% SBE-β-CD in Saline) (add these co-solvents sequentially from left to right, and one by one), clear solution.
For example, if 1 mL of working solution is to be prepared, you can add 100 μL of 62.5 mg/mL clear DMSO stock solution to 900 μL of 20% SBE-β-CD physiological saline solution and mix evenly. Preparation of 20% SBE-β-CD in Saline (4°C，1 week): Dissolve 2 g SBE-β-CD in 10 mL saline to obtain a clear solution. Solubility in Formulation 2: ≥ 6.25 mg/mL (39.77 mM) (saturation unknown) in 10% DMSO + 90% Corn Oil (add these co-solvents sequentially from left to right, and one by one), clear solution. For example, if 1 mL of working solution is to be prepared, you can add 100 μL of 62.5 mg/mL clear DMSO stock solution to 900 μL corn oil and mix evenly. View More
Solubility in Formulation 3: ≥ 3.29 mg/mL (20.93 mM) (saturation unknown) in 10% DMSO + 40% PEG300 + 5% Tween80 + 45% Saline (add these co-solvents sequentially from left to right, and one by one), clear solution. Solubility in Formulation 4: 5 mg/mL (31.81 mM) in PBS (add these co-solvents sequentially from left to right, and one by one), clear solution; with ultrasonication (<60°C). |
| Preparing Stock Solutions | 1 mg | 5 mg | 10 mg | |
| 1 mM | 6.3625 mL | 31.8127 mL | 63.6254 mL | |
| 5 mM | 1.2725 mL | 6.3625 mL | 12.7251 mL | |
| 10 mM | 0.6363 mL | 3.1813 mL | 6.3625 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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