| Size | Price | Stock | Qty |
|---|---|---|---|
| 100mg |
|
||
| Other Sizes |
| Targets |
Glutarylcarnitine does not target a specific receptor but is involved in mitochondrial fatty acid oxidation and organic acid metabolism. As an acylcarnitine, it facilitates the transport of glutarate derivatives into the mitochondrial matrix for beta-oxidation and energy production. Its levels reflect the activity of pathways involved in the metabolism of glutaric acid and related organic acids.
|
|---|---|
| ln Vitro |
Important diagnostic metabolites for tandem mass spectrometry screening of dried blood spots include malonyl- and glutarylcarnitine [1]. Glutarylcarnitine excretion in the urine is a particular biochemical indicator of glutaric acidemia type I (GA-1). For the biochemical diagnosis of type I glutaric acidemia, the excretion of glutarylcarnitine in urine is a useful technique [2].
In vitro, glutarylcarnitine is used as a diagnostic metabolite for malonic aciduria and glutaric aciduria type I. It is monitored in most tandem mass spectrometry newborn screening programmes. The compound's levels in blood or dried blood spots are measured to screen for these metabolic disorders. |
| ln Vivo |
In vivo, glutarylcarnitine is a diagnostic biomarker for glutaric aciduria type I (GA-1) and malonic aciduria. Elevated levels of glutarylcarnitine in the urine or blood are a particular biochemical indicator of glutaric acidemia type I. The compound is also involved in the transport and metabolism of fatty acids in mitochondria.
|
| Enzyme Assay |
For in vitro diagnostic assays, glutarylcarnitine levels are measured in dried blood spots or plasma samples using tandem mass spectrometry (MS/MS). Samples are extracted with methanol containing stable isotope-labeled internal standards. Acylcarnitines are derivatized to butyl esters and analyzed by MS/MS. The concentration of glutarylcarnitine is quantified by comparing the peak area ratio to the internal standard.
|
| Cell Assay |
For in vitro cell-based assays, glutarylcarnitine is typically used as a reference standard or control in metabolic studies. Cells are treated with the compound at various concentrations to study its effects on fatty acid oxidation or to validate analytical methods. Its role in mitochondrial metabolism can be studied in cultured fibroblasts from patients with metabolic disorders.
|
| Animal Protocol |
For in vivo animal experiments, glutarylcarnitine levels are measured in rodent models of metabolic disorders. Blood and tissue samples are collected, and acylcarnitine profiles are analyzed by MS/MS. The compound's levels are used as biomarkers to monitor disease progression or the efficacy of therapeutic interventions in models of glutaric aciduria or other organic acidemias.
|
| ADME/Pharmacokinetics |
Pharmacokinetic data for glutarylcarnitine are limited. As an endogenous metabolite, it is produced and metabolized within the body. Its levels reflect the balance between production from glutaryl-CoA and metabolism via beta-oxidation. The compound has a molecular weight of 275.30 g/mol and formula C₁₂H₂₁NO₆. It is excreted in urine, and elevated levels indicate metabolic dysfunction.
|
| Toxicity/Toxicokinetics |
Toxicological data for glutarylcarnitine are limited, as it is an endogenous metabolite. Elevated levels are associated with metabolic disorders such as glutaric aciduria type I, which can cause neurological damage if untreated. The compound itself is not toxic but serves as a diagnostic marker. It is classified as a research-grade reagent and is not for human therapeutic use.
|
| References | |
| Additional Infomation |
O-Glutaryl-L-carnitine is an O-acyl-L-carnitine, where the acyl group is specifically defined as a glutaryl group. It is a human metabolite functionally related to glutaric acid. O-Glutaryl-L-carnitine has been reported to exist in bovine animals (Bos taurus), and relevant data are available for reference.
Glutarylcarnitine is a research-use only compound and has not been approved for clinical applications. It is a diagnostic metabolite for malonic aciduria and glutaric aciduria type I. Its molecular weight is 275.30, and its formula is C₁₂H₂₁NO₆. The compound is available from various research chemical suppliers for diagnostic and metabolomics research. |
| Molecular Formula |
C12H21NO6
|
|---|---|
| Molecular Weight |
275.29824
|
| Exact Mass |
275.137
|
| CAS # |
102636-82-8
|
| Related CAS # |
Glutarylcarnitine-d9 chloride;Glutarylcarnitine lithium
|
| PubChem CID |
71317118
|
| Appearance |
White to off-white solid powder
|
| LogP |
0.2
|
| Hydrogen Bond Donor Count |
1
|
| Hydrogen Bond Acceptor Count |
6
|
| Rotatable Bond Count |
9
|
| Heavy Atom Count |
19
|
| Complexity |
328
|
| Defined Atom Stereocenter Count |
1
|
| SMILES |
C[N+](C)(C)C[C@@H](CC(=O)[O-])OC(=O)CCCC(=O)O
|
| InChi Key |
NXJAXUYOQLTISD-SECBINFHSA-N
|
| InChi Code |
InChI=1S/C12H21NO6/c1-13(2,3)8-9(7-11(16)17)19-12(18)6-4-5-10(14)15/h9H,4-8H2,1-3H3,(H-,14,15,16,17)/t9-/m1/s1
|
| Chemical Name |
(3R)-3-(4-carboxybutanoyloxy)-4-(trimethylazaniumyl)butanoate
|
| HS Tariff Code |
2934.99.9001
|
| 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)
|
| Solubility (In Vitro) |
DMSO : ~2 mg/mL (~7.26 mM)
|
|---|---|
| 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 | 3.6324 mL | 18.1620 mL | 36.3240 mL | |
| 5 mM | 0.7265 mL | 3.6324 mL | 7.2648 mL | |
| 10 mM | 0.3632 mL | 1.8162 mL | 3.6324 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.
Products are for research use only; We do not sell to patients
Copyright 2020 InvivoChem LLC | All Rights Reserved
