| Size | Price | Stock | Qty |
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| 1mg |
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| Other Sizes |
| Targets |
3-Hydroxyisovalerylcarnitine itself is not a signaling molecule that binds to a biological receptor. Its significance is as a diagnostic marker. It accumulates in the body when the enzymatic activity of biotin-dependent carboxylases (like 3-methylcrotonyl-CoA carboxylase) is impaired. This blocks the leucine catabolic pathway, leading to the buildup of this metabolite, which is then esterified to carnitine for excretion.
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| ln Vitro |
In vitro, the primary use of 3-Hydroxyisovalerylcarnitine is as a chemical standard. It is spiked into control blood or plasma samples to calibrate mass spectrometers for newborn screening assays. There is no reported in vitro biological activity (e.g., enzyme inhibition or receptor activation). It is not used in cell culture to study a therapeutic effect.
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| ln Vivo |
In vivo, 3-Hydroxyisovalerylcarnitine is an accumulating metabolite in patients with specific metabolic disorders. Its levels in blood are used to diagnose and monitor these conditions. In animal models, it is not administered for a therapeutic effect but rather is measured as a marker. When administered to animals, it would likely be rapidly excreted in urine and not produce a specific pharmacodynamic effect.
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| Enzyme Assay |
The compound is not used in receptor binding assays. Its application is strictly analytical. The compound is validated as a calibrator in tandem mass spectrometry (MS/MS). A stock solution is prepared in methanol. It is then diluted with a blank matrix (e.g., bovine blood or plasma) to create a series of calibration standards (e.g., 0.5 to 100 uM). The standards are processed alongside patient samples for quantitative analysis.
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| Cell Assay |
No cell-based assays are performed for this compound. For diagnostic validation, a known concentration of the compound is added to whole blood spotted on filter paper (dried blood spots, DBS). The metabolites are extracted with a methanol solution containing an internal standard (e.g., deuterated 3-Hydroxyisovalerylcarnitine). The extract is analyzed directly by FIA-MS/MS without cell culture.
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| Animal Protocol |
No in vivo animal protocols exist for this compound. For the development of diagnostic assays, the compound may be used to spike blank animal blood (e.g., rat or bovine blood) to serve as quality control material. However, it is not administered to live animals for efficacy or toxicity testing. All in vivo relevance is from its measurement in patient samples.
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| ADME/Pharmacokinetics |
The compound is not a drug and has no PK parameters for human use. It is a water-soluble, endogenous metabolite. Its half-life in circulation is short, as it is filtered by the kidneys and excreted in urine. For research, it is stored at -20degC, protected from light. Stock solutions are typically prepared in methanol or water. The compound is stable under these conditions.
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| Toxicity/Toxicokinetics |
Specific toxicology data is not applicable as it is an endogenous metabolite. At the high levels seen in disease, it is not directly toxic but is a marker of toxicity from other pathways. It is also used as a reagent. Standard laboratory safety precautions should be followed. It may be a skin, eye, and respiratory tract irritant. Avoid inhalation, ingestion, and skin contact.
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| References | |
| Additional Infomation |
3-Hydroxyisovalerylcarnitine is a diagnostic biomarker used in clinical chemistry for newborn screening and metabolic disease monitoring. Elevated levels are indicative of disorders in leucine catabolism or biotin metabolism. It is not a drug and has no therapeutic applications, being strictly an analytical reference standard.
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| Molecular Formula |
C12H23NO5
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|---|---|
| Molecular Weight |
261.31
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| Exact Mass |
261.158
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| CAS # |
99159-87-2
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| PubChem CID |
131668011
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| Appearance |
Solid powder
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| LogP |
0.9
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| Hydrogen Bond Donor Count |
1
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| Rotatable Bond Count |
7
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| Heavy Atom Count |
18
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| Complexity |
301
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| Defined Atom Stereocenter Count |
1
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| SMILES |
CC(CO)CC(OC(C[N+](C)(C)C)CC(=O)[O-])=O
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| InChi Key |
DHRNMNHPSWBJEN-UHFFFAOYSA-N
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| InChi Code |
InChI=1S/C12H23NO5/c1-9(2)6-11(16)18-12(17,7-10(14)15)8-13(3,4)5/h9,17H,6-8H2,1-5H3
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| Chemical Name |
3-hydroxy-3-(3-methylbutanoyloxy)-4-(trimethylazaniumyl)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 Note: This product requires protection from light (avoid light exposure) during transportation and storage. |
| 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 | 3.8269 mL | 19.1344 mL | 38.2687 mL | |
| 5 mM | 0.7654 mL | 3.8269 mL | 7.6537 mL | |
| 10 mM | 0.3827 mL | 1.9134 mL | 3.8269 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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