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| Targets |
Not applicable; N-Isovalerylglycine-d9 is a stable isotope-labeled internal standard for analytical quantification, not a pharmacologically active drug targeting specific biological receptors. The unlabeled parent compound N-isovalerylglycine is an acyl glycine and a metabolite of branched-chain amino acid (leucine) catabolism. It serves as a biomarker for isovaleric acidemia and other organic acidemias, but N-Isovalerylglycine-d9 is used solely as an internal standard in mass spectrometry to quantify unlabeled N-isovalerylglycine in biological samples.
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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 cell-free systems, N-Isovalerylglycine-d9 is used as an internal standard to calibrate GC-MS or LC-MS instruments for acyl glycine analysis. It co-elutes with unlabeled N-isovalerylglycine but is distinguished by its mass shift (M+9), allowing precise quantification by isotope dilution. It is not typically used for testing biological activity in cell-free enzyme assays. The unlabeled N-isovalerylglycine is associated with leucine catabolism and is found in the urine of patients with isovaleric acidemia. The labeled version is used to accurately measure these levels for diagnostic purposes. |
| ln Vivo |
N-Isovalerylglycine-d9 is not intended for therapeutic use but serves as an internal standard in diagnostic and research applications. It is used to accurately quantify the levels of N-isovalerylglycine in urine, plasma, and other biological samples from patients with isovaleric acidemia and other organic acidemias. The unlabeled N-isovalerylglycine is a useful diagnostic tool for the diagnosis of many organic acidemias and mitochondrial fatty acid beta-oxidation defects. The deuterated internal standard is spiked into samples prior to analysis to correct for matrix effects, extraction efficiency, and ionization fluctuations, enabling accurate and reproducible quantification.
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| Enzyme Assay |
For quantitative GC-MS or LC-MS analysis, prepare a stock solution of N-Isovalerylglycine-d9 in methanol or acetonitrile at 1 mg/mL. Spike a known amount (e.g., 10-100 ng/mL) of the deuterated standard into biological samples (urine, plasma, serum). For urine samples, perform liquid-liquid extraction or solid-phase extraction (SPE) to isolate acylglycines. Derivatize extracted samples (e.g., with BSTFA or MTBSTFA for GC-MS) to increase volatility. For GC-MS, separate on a DB-5ms column using a temperature gradient. Detect by electron ionization (EI) MS in selected ion monitoring (SIM) mode. For LC-MS/MS, use a C18 column with 0.1% formic acid in water and acetonitrile, detect by positive ion ESI and MRM. Quantify by isotope dilution using the peak area ratio of unlabeled N-isovalerylglycine to N-Isovalerylglycine-d9 against a calibration curve.
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| Cell Assay |
For cell-based studies, N-Isovalerylglycine-d9 is not typically used in live cell assays. However, for studies of branched-chain amino acid metabolism or organic acidemias, cells (e.g., hepatocytes or fibroblasts from patients with isovaleric acidemia) can be cultured in medium containing labeled leucine or valine to trace the production of N-isovalerylglycine. N-Isovalerylglycine-d9 can be added to cell culture supernatants or lysates as an internal standard prior to LC-MS/MS analysis to accurately quantify the production of unlabeled N-isovalerylglycine. This allows researchers to study the kinetics of leucine catabolism and the effects of drugs or genetic modifications on this pathway.
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| Animal Protocol |
For in vivo diagnostic and research applications, collect urine samples from animal models (e.g., mice with genetic defects in leucine metabolism or mitochondrial fatty acid oxidation) or from patients. Add N-Isovalerylglycine-d9 internal standard (e.g., 10-100 ng/mL) to each urine sample. Perform sample preparation (extraction, derivatization) as described in the assay protocol above. Analyze by GC-MS or LC-MS/MS. Quantify N-isovalerylglycine levels using isotope dilution. Elevated levels of N-isovalerylglycine in urine indicate isovaleric acidemia or other disorders of leucine catabolism. This method is used for diagnosis and for monitoring the efficacy of dietary interventions (e.g., leucine-restricted diets) or drug treatments.
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| ADME/Pharmacokinetics |
N-Isovalerylglycine-d9: Molecular formula C₇H4D₉NO3 (unlabeled C₇H13NO3). Molecular weight: 168.24 g/mol (unlabeled 159.18). Appearance: White to off-white solid. Purity: ≥95% by HPLC; isotopic enrichment: ≥98 atom% D. Solubility: Soluble in DMSO, methanol, and water. Storage: Store powder at -20degC, sealed, protected from light and moisture. In solution, store at -80degC for up to 6 months. Shipping: Room temperature. The compound is also known as N-Iso Valerylglycine-d9, N-(3-Methyl-1-oxobutyl)glycine-d9, Isovalerylglycine-d9. Unlabeled CAS: 24003-63-2. It is a stable isotope-labeled derivative of N-Iso Valerylglycine.
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| Toxicity/Toxicokinetics |
As a stable isotope-labeled internal standard, N-Isovalerylglycine-d9 has low toxicity at typical analytical working concentrations (ng/mL to ug/mL). The unlabeled parent compound N-isovalerylglycine is an endogenous metabolite and is generally regarded as safe at physiological levels, though elevated levels are diagnostic for disease. Standard laboratory safety precautions should be followed when handling the pure compound: wear appropriate personal protective equipment (lab coat, gloves, safety glasses), avoid inhalation of powder and contact with skin/eyes, wash hands thoroughly after handling. Not for human therapeutic use. Always consult the Safety Data Sheet (SDS) for detailed safety information. For research use only.
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| References | |
| Additional Infomation |
N-Isovalerylglycine-d9 is a stable isotope-labeled internal standard for the accurate quantification of N-isovalerylglycine by GC-MS or LC-MS. N-Isovalerylglycine (unlabeled) is an acyl glycine and a metabolite associated with the catabolism of branched-chain amino acids like leucine. It is a useful diagnostic tool for the diagnosis of many organic acidemias and mitochondrial fatty acid beta-oxidation defects, and is found in the urine of patients with isovaleric acidemia, an inborn error of leucine metabolism. The compound is also used as a biomarker for the predisposition for weight gain and obesity. The deuterated version (with nine deuterium atoms) provides a large mass shift (M+9), minimizing isotopic interference and enabling robust quantification in complex biological matrices. The compound is supplied as a reference standard for quantitative analysis, quality control, and related biochemical research applications. For research use only; not for human therapeutic use.
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| Molecular Formula |
C7H4D9NO3
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|---|---|
| Molecular Weight |
168.24
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| Exact Mass |
168.146
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| CAS # |
1330037-21-2
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| Related CAS # |
N-Isovaleroylglycine;16284-60-9
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| PubChem CID |
131668326
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| Appearance |
White to off-white solid powder
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| Density |
1.1±0.1 g/cm3
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| Boiling Point |
371.8±25.0 °C at 760 mmHg
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| Flash Point |
178.7±23.2 °C
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| Vapour Pressure |
0.0±1.8 mmHg at 25°C
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| Index of Refraction |
1.459
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| LogP |
1.5
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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 |
4
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| Heavy Atom Count |
11
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| Complexity |
154
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| Defined Atom Stereocenter Count |
0
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| SMILES |
[2H]C([2H])([2H])C([2H])(C([2H])([2H])[2H])C([2H])([2H])C(=O)NCC(=O)O
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| InChi Key |
ZRQXMKMBBMNNQC-APXHDBETSA-N
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| InChi Code |
InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)/i1D3,2D3,3D2,5D
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| Chemical Name |
2-[[2,2,3,4,4,4-hexadeuterio-3-(trideuteriomethyl)butanoyl]amino]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 |
| 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 | 5.9439 mL | 29.7194 mL | 59.4389 mL | |
| 5 mM | 1.1888 mL | 5.9439 mL | 11.8878 mL | |
| 10 mM | 0.5944 mL | 2.9719 mL | 5.9439 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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