| Size | Price | Stock | Qty |
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| 1mg |
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| 5mg |
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| Other Sizes |
| Targets |
None (metabolite biomarker). Glutarylcarnitine (C5-DC, glutaryl carnitine) is an acylcarnitine species that accumulates in the blood and urine of patients with glutaric aciduria type I (GA-1) due to deficiency of glutaryl-CoA dehydrogenase (GCDH) in the lysine/hydroxylysine/tryptophan catabolism pathway. It also accumulates in malonic aciduria (due to malonyl-CoA decarboxylase deficiency). Glutarylcarnitine itself does not have a therapeutic target or receptor; it is a metabolic byproduct and a diagnostic marker. The deuterated form (d9) is not pharmacologically active; it is used as an internal standard for quantification of the endogenous metabolite by mass spectrometry. The chloride salt (Cl) is used to improve water solubility and solid stability. This compound is not a drug and has no pharmacological activity.
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| ln Vitro |
Drug compounds have included stable heavy isotopes of carbon, hydrogen, and other elements, mostly as tracers for quantification throughout the drug development process. Due to its potential to alter the pharmacokinetic and metabolic characteristics of medications, deuteration has drawn attention[1].
Not applicable. Glutarylcarnitine-d9 chloride is not intended for use in in vitro biological activity assays (e.g., enzyme inhibition, cell proliferation). It is an analytical standard. However, endogenous glutarylcarnitine is a metabolite that is elevated in certain inborn errors of metabolism. In vitro, the non-labeled glutarylcarnitine can be used to spike into biological samples to validate analytical methods. It has no known direct biological activity on receptors or enzymes; it is not cytotoxic to cells at physiological concentrations (0.1-100 uM). In enzyme assays, glutarylcarnitine is not a substrate for most enzymes; it is a product of the conjugation of glutaryl-CoA with carnitine by carnitine acyltransferases. The d9-labeled version is not used in such assays. Therefore, no in vitro activity data are available. The compound's only role is as an internal standard. |
| ln Vivo |
Not applicable. Glutarylcarnitine-d9 chloride is not administered to animals for efficacy studies. It is used as an internal standard to measure endogenous glutarylcarnitine levels in biological samples from animal models of metabolic disorders (e.g., Gcdh knockout mice). In such studies, the d9-labeled compound is not the therapeutic agent; it is added to samples to correct for matrix effects and ionization efficiency during LC-MS/MS analysis. The endogenous glutarylcarnitine level is elevated in GA-1 model mice, and treatment with dietary modifications (e.g., lysine restriction, carnitine supplementation) may reduce its levels. The d9-labeled standard is used to quantify the reduction. No in vivo activity (e.g., efficacy, toxicity) of the labeled compound itself is evaluated, as it is not intended for in vivo administration. The compound is for ex vivo analytical use only.
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| Enzyme Assay |
Not applicable. Glutarylcarnitine-d9 chloride is not used in receptor or enzyme binding assays. As an internal standard, it is used in mass spectrometry-based quantification methods. The standard analytical procedure for quantifying glutarylcarnitine in dried blood spots (DBS) or plasma by LC-MS/MS is as follows: A 3.2 mm punch of a DBS sample is placed in a 96-well plate. 100 uL of extraction solution (methanol containing internal standards, including glutarylcarnitine-d9 at a fixed concentration, typically 0.1-5 uM) is added. The plate is sealed, shaken for 20-30 minutes at room temperature, and then the extract is transferred to a new plate. The sample is injected into an LC-MS/MS system equipped with a C18 column (e.g., 3.0 × 30 mm, 2.6 um). The mobile phase consists of (A) water with 0.1% formic acid and (B) acetonitrile with 0.1% formic acid, using a gradient (e.g., 0-1 min 5% B, 1-2 min 5-95% B, 2-3 min 95% B, 3-3.5 min 95-5% B, 3.5-4 min 5% B). The flow rate is 0.4-0.8 mL/min. The MS/MS is operated in positive electrospray ionization (ESI+) mode with multiple reaction monitoring (MRM). The precursor-to-product ion transition for glutarylcarnitine-d9 is m/z 329.2 → 85.2 (or similar, depending on the instrument). The transition for the non-labeled compound is m/z 320.2 → 85.2 (for C5-DC). The internal standard (d9) compensates for variations in extraction efficiency and ionization. The ratio of the analyte peak area to the internal standard peak area is used for quantification. A calibration curve is prepared by spiking known concentrations of non-labeled glutarylcarnitine into blank matrix with a fixed concentration of d9 internal standard. The linear range is typically 0.1-100 uM. The method is validated for accuracy, precision, and linearity. This is the only "assay" in which the compound is used. No non-cellular binding assays are performed.
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| Cell Assay |
Not applicable. The primary use of glutarylcarnitine-d9 chloride is as an internal standard in LC-MS/MS analysis of biological samples. It is not used in cell-based experiments (e.g., cell culture, cell proliferation, toxicity) because it is not a drug; it is only an analytical reagent. However, researchers could theoretically use the compound to spike into cell culture medium to serve as a control for method development, but this is not typical. There are no standard protocols for in vitro cell-based assays using this compound. The compound is not cytotoxic, but no formal studies exist. For cell-based metabolomics, cells (e.g., hepatocytes, fibroblasts) are cultured, and the medium is collected, then processed for acylcarnitine analysis by LC-MS/MS, and the d9 compound is added during extraction as an internal standard. This is an analytical workflow, not a biological assay. For example, human fibroblasts from GA-1 patients are cultured, and lysates are extracted with methanol containing glutarylcarnitine-d9 (0.1-1 uM). The extract is analyzed by LC-MS/MS to quantify endogenous glutarylcarnitine. The d9 compound corrects for extraction and ionization efficiency. This is not a "cell-based assay" in the sense of pharmacology. Therefore, no detailed protocol is provided.
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| Animal Protocol |
For in vivo studies, glutarylcarnitine-d9 is not administered to animals. However, the non-labeled endogenous glutarylcarnitine is measured in animal models of glutaric aciduria type I (GA-1). To quantify glutarylcarnitine in mouse plasma, the following sample preparation protocol is used: Blood (50-100 uL) is collected from the tail vein or via cardiac puncture into EDTA-coated tubes. Plasma is separated by centrifugation (2,000 × g, 10 min, 4degC). A 25-50 uL aliquot of plasma is mixed with 150-200 uL of methanol containing the internal standard (glutarylcarnitine-d9 at a concentration of 0.1-1 uM). The mixture is vortexed, incubated at 4degC for 10-20 min to precipitate proteins, and then centrifuged at 15,000 × g for 10 min. The supernatant is transferred to an LC-MS/MS vial and analyzed as described in Section 5. The concentration of endogenous glutarylcarnitine is calculated using the internal standard and a calibration curve. No animal dosing is involved. The d9 compound is not used for treatment; it is only for analysis. Therefore, no in vivo animal experimental protocol for the compound itself as a drug is applicable. All animal procedures require IACUC approval.
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| ADME/Pharmacokinetics |
Not applicable. Glutarylcarnitine-d9 chloride is not administered in vivo; it is an analytical internal standard. The pharmacokinetics of exogenous glutarylcarnitine have not been studied, but if given intravenously, it would be rapidly cleared by the kidneys. The non-labeled glutarylcarnitine is an endogenous metabolite; its plasma concentration in healthy individuals is <0.5 uM. In GA-1 patients, it is elevated >10 uM. The d9-labeled version has identical chemical properties and is used only as a stable isotope internal standard. The compound is stable in methanol or aqueous solution for months at -20degC. No ADME (absorption, distribution, metabolism, excretion) studies are conducted on the labeled compound. The TFA salt is not used; this product is chloride salt. The compound is not a drug, and PK data are not available.
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| Toxicity/Toxicokinetics |
No toxicity data are available for glutarylcarnitine-d9 chloride. The non-labeled glutarylcarnitine is an endogenous metabolite that accumulates in metabolic diseases, but it is not considered toxic in itself; rather, its accumulation is a marker of the underlying enzymatic defect. High levels of glutarylcarnitine are associated with neurologic damage in GA-1 (through secondary effects), but the compound itself is not directly toxic. The d9-labeled version is chemically identical except for the deuterium atoms, which are non-radioactive and stable. The compound is not administered to animals or humans in therapeutic doses; it is used only as an analytical reagent in small amounts (ug or mg). Therefore, toxicity is not relevant. The chloride salt is not toxic. Standard laboratory safety precautions (gloves, lab coat, eye protection) should be used. The product is for research use only, not for human or animal use. It is not a drug.
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| References |
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| Additional Infomation |
Glutarylcarnitine (C5-DC) is an acylcarnitine species with the chemical formula C12H21NO6. It is formed by the esterification of glutaric acid with carnitine. Glutaric acid is an intermediate in the catabolism of lysine, hydroxylysine, and tryptophan. In glutaric aciduria type I (GA-1), a rare autosomal recessive disorder caused by deficiency of glutaryl-CoA dehydrogenase (GCDH), the metabolism of glutaryl-CoA is blocked, leading to accumulation of glutaric acid, 3-hydroxyglutaric acid, and glutarylcarnitine. GA-1 presents with macrocephaly, acute encephalopathic crises, and striatal injury. Glutarylcarnitine is a key biomarker used in newborn screening by tandem mass spectrometry (MS/MS) to detect GA-1 and malonic aciduria. The deuterated form (d9) is used as an internal standard to improve the accuracy and precision of quantitative MS assays. The compound is supplied as a chloride salt for enhanced solubility. This product is not a drug; it is an analytical standard for research and clinical diagnostic use. It is for research use only and not intended for therapeutic application.
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| Molecular Formula |
C12H13D9CLNO6
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| Molecular Weight |
320.81
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| Related CAS # |
Glutarylcarnitine;102636-82-8
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| Appearance |
White to off-white ointment
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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 | 3.1171 mL | 15.5855 mL | 31.1711 mL | |
| 5 mM | 0.6234 mL | 3.1171 mL | 6.2342 mL | |
| 10 mM | 0.3117 mL | 1.5586 mL | 3.1171 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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