| Size | Price | Stock | Qty |
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| 50mg |
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| 100mg |
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| Other Sizes |
| Targets |
Glutaric acid-d4 shares the same biological targets as its non-deuterated parent compound. It interacts with enzymes involved in lysine and tryptophan catabolism, particularly glutaryl-CoA dehydrogenase (GCDH), the enzyme deficient in Glutaric Aciduria Type I. Glutaric acid accumulates in the mitochondria and central nervous system when GCDH is defective, leading to neurological damage. The compound also affects pericyte contractility and migration, suggesting involvement in vascular regulation.
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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].
In vitro, glutaric acid (non-deuterated) is used to study metabolic pathways involving lysine and tryptophan degradation. It affects pericyte contractility and migration in cell culture models, which may contribute to blood-brain barrier dysfunction in GA1. Glutaric acid also influences cellular metabolism and mitochondrial function. The deuterated version (Glutaric acid-d4) is not used for activity studies but serves as an internal standard for quantification of glutaric acid in biological samples such as urine, plasma, and dried blood spots. |
| ln Vivo |
In vivo, glutaric acid (non-deuterated) accumulates in patients with Glutaric Aciduria Type I (GA1), leading to neurological damage including striatal injury and movement disorders. In animal models, administration of glutaric acid induces neurotoxicity and oxidative stress, mimicking the pathophysiology of GA1. Glutaric acid-d4 is not used for in vivo activity studies; instead, it is used as an analytical standard to quantify endogenous glutaric acid levels in patient samples for diagnostic and monitoring purposes.
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| Enzyme Assay |
For non-cellular assays (analytical quantification), Glutaric acid-d4 is prepared as a stock solution in methanol (1 mg/mL). For LC-MS/MS analysis, a calibration curve for glutaric acid is prepared in human urine or plasma (0.1-1000 ng/mL) with a fixed concentration of glutaric acid-d4 (e.g., 50 ng/mL). Samples are extracted using protein precipitation with acetonitrile or ethyl acetate, followed by centrifugation. The supernatant is injected onto a C18 column with a mobile phase of 0.1% formic acid in water and acetonitrile (gradient elution). Ions are monitored in MRM mode: m/z 136.1 → 74.1 for glutaric acid-d4, m/z 132.1 → 70.1 for non-deuterated glutaric acid.
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| Cell Assay |
For cell-based assays, glutaric acid studies use primary neurons or astrocytes from rodent brain. Cells are seeded in 6-well plates (1×10⁶ cells/well) in Neurobasal medium with B27 supplement. After 7 days of differentiation, cells are treated with glutaric acid (0.1-5 mM) for 24-72 hours. Cell viability is assessed by MTT or LDH release assays. Oxidative stress is evaluated by measuring ROS production (DCFH-DA, 10 uM, 30 min), lipid peroxidation (MDA levels), and antioxidant enzyme activities (SOD, catalase). Mitochondrial membrane potential is assessed using JC-1 staining. Glutaric acid-d4 can be used to quantify intracellular glutaric acid levels in treated cells by LC-MS.
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| Animal Protocol |
For in vivo animal experiments, Glutaric Aciduria Type I (GA1) models are used. Male Wistar rats (7 days old) are administered glutaric acid (1.5-3.0 umol/g body weight) subcutaneously twice daily for 3-5 days to induce neurotoxicity. Alternatively, GCDH knockout mice are used. Neurological assessments include open field tests, rotarod performance, and Morris water maze. Brain tissues (striatum, cortex, hippocampus) are collected for histological analysis (H&E, Fluoro-Jade B staining), measurement of oxidative stress markers (GSH, TBARS), and quantification of glutaric acid levels by LC-MS/MS using glutaric acid-d4 as internal standard.
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| ADME/Pharmacokinetics |
Glutaric acid-d4 has a molecular weight of 136.14 and a density of 1.3+/-0.1 g/cm3, with a boiling point of 302.9+/-15.0degC. The compound is a white to off-white solid powder, soluble in DMSO and water. It has a logP of -1.04, indicating high hydrophilicity. The deuterium atoms at positions 2 and 4 provide a mass shift of +4 Da, enabling clear differentiation from non-deuterated glutaric acid in mass spectrometry. The compound should be stored as a powder at -20degC, protected from light and moisture, with stability for up to 3 years.
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| Toxicity/Toxicokinetics |
Glutaric acid-d4 is a stable isotope-labeled compound with low toxicity at analytical concentrations (ng-ug per sample). The non-deuterated parent compound, glutaric acid, at high doses can cause neurotoxicity and oxidative stress. However, when used as an internal standard, the amount is negligible and poses no toxicity risk. Standard laboratory safety precautions for handling organic compounds should be followed. The compound is non-radioactive and poses no radiation hazard.
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| References |
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| Additional Infomation |
Glutaric acid-d4 is an analytical standard and research tool, not an approved drug. It has not undergone clinical trials for therapeutic use. Its primary application is as an internal standard for the quantitative analysis of glutaric acid in biological samples by LC-MS/MS. This is clinically relevant for the diagnosis and monitoring of Glutaric Aciduria Type I (GA1), a rare inherited metabolic disorder. The compound is also used in metabolomics research to study lysine and tryptophan metabolism. It is available for research use only.
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| Molecular Formula |
C5H4D4O4
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|---|---|
| Molecular Weight |
136.14
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| Exact Mass |
136.067
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| CAS # |
19136-99-3
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| Related CAS # |
Glutaric acid;110-94-1
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| PubChem CID |
53952054
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| Appearance |
White to off-white solid powder
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| Density |
1.3±0.1 g/cm3
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| Boiling Point |
302.9±15.0 °C at 760 mmHg
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| Flash Point |
151.2±16.9 °C
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| Vapour Pressure |
0.0±1.4 mmHg at 25°C
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| Index of Refraction |
1.477
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| LogP |
-1.04
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| Hydrogen Bond Donor Count |
2
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| Hydrogen Bond Acceptor Count |
4
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| Rotatable Bond Count |
4
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| Heavy Atom Count |
9
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| Complexity |
104
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| Defined Atom Stereocenter Count |
0
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| SMILES |
[2H]C([2H])(CC([2H])([2H])C(=O)O)C(=O)O
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| InChi Key |
JFCQEDHGNNZCLN-RRVWJQJTSA-N
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| InChi Code |
InChI=1S/C5H8O4/c6-4(7)2-1-3-5(8)9/h1-3H2,(H,6,7)(H,8,9)/i2D2,3D2
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| Chemical Name |
2,2,4,4-tetradeuteriopentanedioic 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 |
| 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 | 7.3454 mL | 36.7269 mL | 73.4538 mL | |
| 5 mM | 1.4691 mL | 7.3454 mL | 14.6908 mL | |
| 10 mM | 0.7345 mL | 3.6727 mL | 7.3454 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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