| Size | Price | Stock | Qty |
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| 5mg |
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| 10mg |
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| Other Sizes |
| Targets |
Hexanoylglycine-d2, as an isotopically labeled internal standard, does not target specific biological receptors. The non-labeled parent compound, hexanoylglycine, is an acyl glycine metabolite that is produced when medium-chain fatty acids are conjugated with glycine. This conjugation reaction is catalyzed by the enzyme glycine N-acyltransferase (GLYAT) in the mitochondria. Elevated levels of hexanoylglycine are a marker for impaired fatty acid oxidation, particularly medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. In this disorder, the accumulation of medium-chain acyl-CoA esters leads to increased formation of hexanoylglycine via glycine conjugation. The deuterated version is used solely as an analytical standard for diagnostic applications and does not engage in biological interactions for therapeutic purposes.
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| ln Vitro |
Drug compounds have included stable heavy isotopes of carbon, hydrogen, and other elements, mostly as quantitative tracers while the drugs were being developed. Because deuteration may have an effect on a drug's pharmacokinetics and metabolic properties, it is a cause for concern [1].
As a deuterium-labeled internal standard, hexanoylglycine-d2 itself does not possess intrinsic in vitro biological activity. It is used exclusively for analytical quantification purposes, particularly in the detection of metabolic disorders using mass spectrometry. Hexanoylglycine-d2 is used as an internal standard for the quantification of hexanoylglycine in biological samples such as urine, plasma, or dried blood spots. This is critical for the diagnosis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, a disorder of fatty acid oxidation. In vitro, the concentration of hexanoylglycine can be measured in cell lysates or culture supernatants. The compound is added to samples at a known concentration prior to extraction and analysis to correct for variations in sample preparation and instrument performance. |
| ln Vivo |
Hexanoylglycine-d2 does not exhibit in vivo biological activity because it is an analytical standard. The non-labeled hexanoylglycine is a naturally occurring metabolite found in the urine and plasma of healthy individuals. In patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, the concentration of hexanoylglycine in urine is elevated and serves as a diagnostic biomarker. MCAD deficiency is an autosomal recessive disorder that impairs the metabolism of medium-chain fatty acids, leading to hypoketotic hypoglycemia, vomiting, lethargy, and in severe cases, coma or death. Early detection of elevated hexanoylglycine levels by newborn screening using tandem mass spectrometry can lead to early intervention and improved outcomes. The deuterated internal standard is used to enable precise quantification of hexanoylglycine in screening programs.
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| Enzyme Assay |
A typical non-cellular protocol for using hexanoylglycine-d2 as an internal standard involves its inclusion in the sample preparation workflow for LC-MS or GC-MS analysis. A stock solution of the internal standard (1 mg/mL) is prepared in methanol or acetonitrile. For urine samples, 10 uL of urine is diluted with 90 uL of water in a microcentrifuge tube. Then, 10 uL of the internal standard solution (diluted to a working concentration of 1 ug/mL) is added to achieve a final concentration of 100 ng/mL. For dried blood spots (DBS), a 3 mm punch is placed in a microcentrifuge tube, and 100 uL of methanol containing the internal standard is added. The tube is vortexed for 30 minutes at room temperature. After extraction, the sample is centrifuged, and the supernatant is transferred to an autosampler vial. The sample (5-10 uL) is injected onto an LC-MS/MS system or derivatized for GC-MS analysis. Quantification is achieved by calculating the peak area ratio of the analyte to the internal standard against a calibration curve.
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| Cell Assay |
A typical in vitro cellular protocol for using hexanoylglycine-d2 as an internal standard involves the quantification of hexanoylglycine in cultured hepatocytes. Primary human hepatocytes or HepG2 cells are seeded in 6-well plates at a density of 1×10⁶ cells/well and cultured in appropriate medium for 24 hours. After treatment with test compounds (e.g., fatty acids or metabolic modulators), the culture medium is collected. Cells are washed twice with ice-cold PBS and harvested by scraping. Cells are pelleted by centrifugation (500 g, 5 minutes). For the analysis of hexanoylglycine, 100 uL of culture medium or cell lysate is mixed with 10 uL of internal standard solution (1 ug/mL) and 300 uL of acetonitrile. After centrifugation, the supernatant is analyzed by LC-MS/MS. The concentration of hexanoylglycine is normalized to total protein content for cell lysates. The internal standard corrects for extraction efficiency and matrix effects.
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| Animal Protocol |
Hexanoylglycine-d2 is typically used as an internal standard in animal studies for the quantification of hexanoylglycine in plasma, urine, and tissues. In a typical protocol, male C57BL/6 mice (8-10 weeks old, 20-25 g) are used. Mice are fasted for 12 hours before the experiment to induce fatty acid oxidation. Blood samples (50-100 uL) are collected via the tail vein into EDTA-coated tubes at various time points (0, 2, 4, 6, 12, 24 hours) after administration of a test compound (e.g., a medium-chain triglyceride load). Urine samples are collected using metabolic cages over a 24-hour period. Plasma is separated by centrifugation (2,000 g, 10 minutes, 4degC). For extraction, 50 uL of plasma or urine is mixed with 10 uL of internal standard solution (1 ug/mL) and 150 uL of acetonitrile. After centrifugation, the supernatant is analyzed by LC-MS/MS. The concentration of hexanoylglycine is quantified using a calibration curve prepared with the same internal standard.
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| ADME/Pharmacokinetics |
As an analytical internal standard, hexanoylglycine-d2 is not characterized by typical pharmacokinetic parameters. It is not intended to be administered as a test article for PK studies. The non-labeled hexanoylglycine is an endogenous metabolite that is normally present at low concentrations in plasma and urine. In healthy individuals, urinary hexanoylglycine levels are typically below the detection limit or very low. In patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, urinary hexanoylglycine levels can be significantly elevated (e.g., >100 mmol/mol creatinine). Plasma concentrations may also be elevated. The compound is formed from medium-chain fatty acids via the glycine conjugation pathway. The deuterium-labeled version, when used as an internal standard, is added exogenously to samples and does not contribute to the in vivo PK profile of any test compound.
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| Toxicity/Toxicokinetics |
Toxicity data specific to hexanoylglycine-d2 are not available. The non-labeled hexanoylglycine is an endogenous metabolite and is generally considered non-toxic at physiological concentrations. The compound is used as a diagnostic marker, not as a therapeutic agent. Standard laboratory safety precautions should be followed when handling the compound, including the use of gloves, lab coats, and safety glasses. Avoid inhalation, ingestion, and direct skin or eye contact. The compound should be stored at -20degC in a tightly sealed container, protected from light and moisture. It is intended for research use only and should not be used in humans or animals for therapeutic or diagnostic purposes. However, it is important to note that elevated levels of hexanoylglycine in the body indicate a potentially life-threatening metabolic disorder (MCAD deficiency), not toxicity of the compound itself.
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| References | |
| Additional Infomation |
Hexanoylglycine-d2 (CAS# 1256842-52-0) is a stable isotope-labeled compound with a molecular weight of 175.22. The molecular formula is C8H13D2NO3, and it is also known as N-hexanoylglycine-2,2-d2, N-n-caproylglycine, and hexanoylaminoacetic acid. The isotopic purity is typically greater than 98%, and the chemical purity is ≥98%. The compound is supplied as a solid and should be stored at -20degC. Hexanoylglycine-d2 is primarily used as an internal standard for the quantification of hexanoylglycine by GC- or LC-MS. Hexanoylglycine is an acyl glycine metabolite that is a diagnostic marker for medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, a fatty acid oxidation disorder. The deuterated version is essential for precise quantification in diagnostic and research applications. This product is for research use only and is not approved for clinical diagnostic use.
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| Molecular Formula |
C8H13D2NO3
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| Molecular Weight |
175.22
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| Exact Mass |
175.118
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| CAS # |
1256842-52-0
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| Related CAS # |
Hexanoylglycine;24003-67-6
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| PubChem CID |
131701120
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| Appearance |
White to off-white solid powder
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| LogP |
1.158
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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 |
6
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| Heavy Atom Count |
12
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| Complexity |
156
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| Defined Atom Stereocenter Count |
0
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| SMILES |
[2H][N+]([2H])(CC(=O)O)C(=O)CCCCC
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| InChi Key |
UPCKIPHSXMXJOX-ZSJDYOACSA-O
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| InChi Code |
InChI=1S/C8H15NO3/c1-2-3-4-5-7(10)9-6-8(11)12/h2-6H2,1H3,(H,9,10)(H,11,12)/p+1/i/hD2
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| Chemical Name |
carboxymethyl-dideuterio-hexanoylazanium
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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 | 5.7071 mL | 28.5356 mL | 57.0711 mL | |
| 5 mM | 1.1414 mL | 5.7071 mL | 11.4142 mL | |
| 10 mM | 0.5707 mL | 2.8536 mL | 5.7071 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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