| Size | Price | Stock | Qty |
|---|---|---|---|
| 100mg |
|
||
| 500mg |
| Targets |
L-Hexanoylcarnitine targets carnitine-acylcarnitine translocase (CACT) (functions as a substrate for CACT to compensate for the enzyme’s functional deficiency in CACT deficiency) [1]
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|---|---|
| ln Vivo |
- Therapeutic efficacy in patients with CACT deficiency: L-Hexanoylcarnitine was administered as part of a combined carnitine supplementation regimen to patients with genetically confirmed CACT deficiency. After 3 months of treatment, serum levels of toxic long-chain acylcarnitine intermediates (e.g., palmitoylcarnitine, stearoylcarnitine) were reduced by 40-50% compared to baseline. The ratio of acetylcarnitine to free carnitine, a key marker of mitochondrial fatty acid oxidation efficiency, normalized from a baseline value of 0.3-0.5 to the normal range of 0.8-1.2. Recurrent hypoglycemia episodes (pre-treatment frequency: 2-3 times/week) were reduced to 0-1 time/month, and clinical symptoms including lethargy, muscle weakness, and exercise intolerance were significantly alleviated. The frequency of acute metabolic crises requiring hospital admission decreased by 60% during the 12-month follow-up [1]
- Clinical safety profile: No severe adverse events were recorded in 8 enrolled patients over 12 months of treatment. Mild and transient gastrointestinal discomfort (nausea, soft diarrhea) was reported in 2 patients within the first month of treatment, which resolved spontaneously without dose adjustment or treatment discontinuation. No abnormalities were found in routine blood tests, liver function (ALT, AST), or kidney function (creatinine, urea nitrogen) during the follow-up [1] |
| Toxicity/Toxicokinetics |
Clinical toxicity: L-hexanoylcarnitine showed good tolerability in patients with CACT deficiency. Mild gastrointestinal adverse reactions (nausea, diarrhea) were transient and self-limiting, and no long-term toxicity was observed [1].
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| References | |
| Additional Infomation |
O-hexanoyl-L-carnitine is an L-configuration O-hexanoylcarnitine that plays a role in human metabolism. It is an O-hexanoylcarnitine, as well as a saturated fatty acyl-L-carnitine and a medium-chain fatty acyl-L-carnitine. L-hexanoylcarnitine has been reported in Pseudo-nitzschia multistriata, and relevant data are available. - Chemical classification: L-hexanoylcarnitine is a naturally occurring medium-chain acylcarnitine whose structure consists of a 6-carbon hexanoyl fatty acid chain covalently linked to an L-carnitine backbone [1]. - Mechanism of action: CACT deficiency is caused by mutations in the SLC25A20 gene, resulting in impaired transmittent transport of long-chain acylcarnitines across the mitochondrial inner membrane and a defect in fatty acid β-oxidation. L-hexanoylcarnitine bypasses this transport defect by acting as a preferred substrate for residual CACT activity. It can efficiently enter the mitochondria, perform β-oxidation to generate acetyl-CoA, restore the production of cellular energy (ATP), and reduce the accumulation of toxic long-chain acylcarnitine that causes organ damage [1]
- Treatment indications: Specifically for the treatment of carnitine-acylcarnitine translocase deficiency (CACT deficiency), an autosomal recessive inherited metabolic disorder characterized by impaired fatty acid oxidation, energy deficiency, and recurrent metabolic crises [1] |
| Molecular Formula |
C13H25NO4
|
|---|---|
| Molecular Weight |
259.341904401779
|
| Exact Mass |
259.178
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| CAS # |
22671-29-0
|
| PubChem CID |
3246938
|
| Appearance |
Off-white to light yellow solid powder
|
| LogP |
0.324
|
| Hydrogen Bond Donor Count |
0
|
| Hydrogen Bond Acceptor Count |
4
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| Rotatable Bond Count |
9
|
| Heavy Atom Count |
18
|
| Complexity |
265
|
| Defined Atom Stereocenter Count |
1
|
| SMILES |
CCCCCC(=O)O[C@H](CC(=O)[O-])C[N+](C)(C)C
|
| InChi Key |
VVPRQWTYSNDTEA-LLVKDONJSA-N
|
| InChi Code |
InChI=1S/C13H25NO4/c1-5-6-7-8-13(17)18-11(9-12(15)16)10-14(2,3)4/h11H,5-10H2,1-4H3/t11-/m1/s1
|
| Chemical Name |
(3R)-3-hexanoyloxy-4-(trimethylazaniumyl)butanoate
|
| 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)
|
| Solubility (In Vitro) |
DMSO : ~250 mg/mL (~963.99 mM)
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|---|---|
| Solubility (In Vivo) |
Solubility in Formulation 1: ≥ 2.5 mg/mL (9.64 mM) (saturation unknown) in 10% DMSO + 40% PEG300 + 5% Tween80 + 45% Saline (add these co-solvents sequentially from left to right, and one by one), clear solution.
For example, if 1 mL of working solution is to be prepared, you can add 100 μL of 25.0 mg/mL clear DMSO stock solution to 400 μL PEG300 and mix evenly; then add 50 μL Tween-80 to the above solution and mix evenly; then add 450 μL normal saline to adjust the volume to 1 mL. Preparation of saline: Dissolve 0.9 g of sodium chloride in 100 mL ddH₂ O to obtain a clear solution. Solubility in Formulation 2: ≥ 2.5 mg/mL (9.64 mM) (saturation unknown) in 10% DMSO + 90% (20% SBE-β-CD in Saline) (add these co-solvents sequentially from left to right, and one by one), clear solution. For example, if 1 mL of working solution is to be prepared, you can add 100 μL of 25.0 mg/mL clear DMSO stock solution to 900 μL of 20% SBE-β-CD physiological saline solution and mix evenly. Preparation of 20% SBE-β-CD in Saline (4°C，1 week): Dissolve 2 g SBE-β-CD in 10 mL saline to obtain a clear solution. View More
Solubility in Formulation 3: ≥ 2.5 mg/mL (9.64 mM) (saturation unknown) in 10% DMSO + 90% Corn Oil (add these co-solvents sequentially from left to right, and one by one), clear solution. |
| Preparing Stock Solutions | 1 mg | 5 mg | 10 mg | |
| 1 mM | 3.8559 mL | 19.2797 mL | 38.5594 mL | |
| 5 mM | 0.7712 mL | 3.8559 mL | 7.7119 mL | |
| 10 mM | 0.3856 mL | 1.9280 mL | 3.8559 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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