3-Hydroxyisovalerylcarnitine

Cat. No.: HY-N11200: Data Sheet
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Handling Instructions
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3-Hydroxyisovalerylcarnitine is a carnitine derivative. 3-Hydroxyisovaleryl carnitine in plasma may serve as a novel biomarker of biotin deficiency in humans, with its concentration increasing with biotin deficiency. Biotin deficiency reduces the activity of biotin-dependent 3-methylcrotonyl-CoA carboxylase, hinders the conversion of 3-methylcrotonyl-CoA into 3-methylglutaconyl-CoA, and impairs the leucine catabolism pathway; resulting in plasma 3-Hydroxyisovalerylcarnitine concentrations rise.

For research use only. We do not sell to patients.

3-Hydroxyisovalerylcarnitine Chemical Structure

CAS No. : 99159-87-2

Size	Stock	Quantity
1 mg	In-stock	Estimated Time of Arrival: December 31
5 mg	Get quote
10 mg	Get quote

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Other Forms of 3-Hydroxyisovalerylcarnitine:

3-Hydroxyisovalerylcarnitine (Standard) Get quote

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Biological Activity
Purity & Documentation
References
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Description

In Vitro

3-Hydroxyisovalerylcarnitine is a diagnostic marker for multiple carboxylase deficiency (MCD). 3-Hydroxyisovalerylcarnitine accumulates with decreased activity of various carboxylases, and can be used to diagnose organic acidemia in newborns^[2].

MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

Molecular Weight

261.31

Formula

C₁₂H₂₃NO₅

CAS No.

99159-87-2

Appearance

Solid

Color

White to off-white

SMILES

CC(O)(CC(O[C@@H](C[N+](C)(C)C)CC([O-])=O)=O)C

Structure Classification

Others

Initial Source

Microorganisms

Suillus laricinus

Shipping

Room temperature in continental US; may vary elsewhere.

Storage

-20°C, protect from light

*In solvent : -80°C, 6 months; -20°C, 1 month (protect from light)

Solvent & Solubility

In Vitro:

DMSO : ≥ 50 mg/mL (191.34 mM; Hygroscopic DMSO has a significant impact on the solubility of product, please use newly opened DMSO)

H₂O : 1 mg/mL (3.83 mM; Need ultrasonic and warming)

*"≥" means soluble, but saturation unknown.

Preparing
Stock Solutions

Concentration Solvent Mass	1 mg	5 mg	10 mg

1 mM	3.8269 mL	19.1344 mL	38.2687 mL
5 mM	0.7654 mL	3.8269 mL	7.6537 mL
10 mM	0.3827 mL	1.9134 mL	3.8269 mL

View the Complete Stock Solution Preparation Table

* Please refer to the solubility information to select the appropriate solvent. Once prepared, please aliquot and store the solution to prevent product inactivation from repeated freeze-thaw cycles.
Storage method and period of stock solution: -80°C, 6 months; -20°C, 1 month (protect from light). When stored at -80°C, please use it within 6 months. When stored at -20°C, please use it within 1 month.

* Note: If you choose water as the stock solution, please dilute it to the working solution, then filter and sterilize it with a 0.22 μm filter before use.

Molarity Calculator
Dilution Calculator

Mass (g) = Concentration (mol/L) × Volume (L) × Molecular Weight (g/mol)

Concentration _(start) × Volume _(start) = Concentration _(final) × Volume _(final)

This equation is commonly abbreviated as: C₁V₁ = C₂V₂

Concentration _(start)

Volume _(start)

Concentration _(final)

Volume _(final)

In Vivo:

Select the appropriate dissolution method based on your experimental animal and administration route.

For the following dissolution methods, please ensure to first prepare a clear stock solution using an In Vitro approach and then sequentially add co-solvents:
To ensure reliable experimental results, the clarified stock solution can be appropriately stored based on storage conditions. As for the working solution for in vivo experiments, it is recommended to prepare freshly and use it on the same day.
The percentages shown for the solvents indicate their volumetric ratio in the final prepared solution. If precipitation or phase separation occurs during preparation, heat and/or sonication can be used to aid dissolution.

Protocol 1

Add each solvent one by one: 10% DMSO 40% PEG300 5% Tween-80 45% Saline
Solubility: ≥ 1.25 mg/mL (4.78 mM); Clear solution

This protocol yields a clear solution of ≥ 1.25 mg/mL (saturation unknown).
Taking 1 mL working solution as an example, add 100 μL DMSO stock solution (12.5 mg/mL) to 400 μL PEG300, and mix evenly; then add 50 μL Tween-80 and mix evenly; then add 450 μL Saline to adjust the volume to 1 mL.
Preparation of Saline: Dissolve 0.9 g sodium chloride in ddH₂O and dilute to 100 mL to obtain a clear Saline solution.
Protocol 2

Add each solvent one by one: 10% DMSO 90% (20% SBE-β-CD in Saline)
Solubility: ≥ 1.25 mg/mL (4.78 mM); Clear solution

This protocol yields a clear solution of ≥ 1.25 mg/mL (saturation unknown).
Taking 1 mL working solution as an example, add 100 μL DMSO stock solution (12.5 mg/mL) to 900 μL 20% SBE-β-CD in Saline, and mix evenly.
Preparation of 20% SBE-β-CD in Saline (4°C, storage for one week): 2 g SBE-β-CD powder is dissolved in 10 mL Saline, completely dissolve until clear.
Protocol 3

Add each solvent one by one: 10% DMSO 90% Corn Oil
Solubility: 1.25 mg/mL (4.78 mM); Suspended solution; Need ultrasonic

This protocol yields a suspended solution of 1.25 mg/mL. Suspended solution can be used for oral and intraperitoneal injection.
Taking 1 mL working solution as an example, add 100 μL DMSO stock solution (12.5 mg/mL) to 900 μL Corn oil, and mix evenly.

In Vivo Dissolution Calculator

Please enter the basic information of animal experiments:

Dosage

mg/kg

Animal weight
(per animal)

Dosing volume
(per animal)

μL

Number of animals

Recommended: Prepare an additional quantity of animals to account for potential losses during experiments.

Please enter your animal formula composition:

DMSO +

Tween-80 +

Saline

Recommended: Keep the proportion of DMSO in working solution below 2% if your animal is weak.

The co-solvents required include: DMSO, . All of co-solvents are available by MedChemExpress (MCE). , Tween 80. All of co-solvents are available by MedChemExpress (MCE).

Calculation results:

Working solution concentration: mg/mL

Method for preparing stock solution: mg drug dissolved in μL DMSO (Stock solution concentration: mg/mL).

*In solvent : -80°C, 6 months; -20°C, 1 month (protect from light)

The concentration of the stock solution you require exceeds the measured solubility. The following solution is for reference only. If necessary, please contact MedChemExpress (MCE).

Method for preparing in vivo working solution for animal experiments: Take μL DMSO stock solution, add μL . μL , mix evenly, next add μL Tween 80, mix evenly, then add μL Saline.

If the continuous dosing period exceeds half a month, please choose this protocol carefully.

Please ensure that the stock solution in the first step is dissolved to a clear state, and add co-solvents in sequence. You can use ultrasonic heating (ultrasonic cleaner, recommended frequency 20-40 kHz), vortexing, etc. to assist dissolution.

Purity & Documentation

Purity: 99.4%

Select Batch:

Data Sheet (273 KB) SDS (393 KB)

COA (249 KB)

Handling Instructions (2659 KB)

References

[1]. Horvath TD, et al. Quantitative measurement of plasma 3-hydroxyisovaleryl carnitine by LC-MS/MS as a novel biomarker of biotin status in humans. Anal Chem. 2010 May 15;82(10):4140-4. [Content Brief]

[2]. Maeda Y, et al., Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2008 Jul 15;870(2):154-9. [Content Brief]

Complete Stock Solution Preparation Table

Optional Solvent	Concentration Solvent Mass	1 mg	5 mg	10 mg	25 mg

H₂O / DMSO	1 mM	3.8269 mL	19.1344 mL	38.2687 mL	95.6718 mL
DMSO	5 mM	0.7654 mL	3.8269 mL	7.6537 mL	19.1344 mL
	10 mM	0.3827 mL	1.9134 mL	3.8269 mL	9.5672 mL
	15 mM	0.2551 mL	1.2756 mL	2.5512 mL	6.3781 mL
	20 mM	0.1913 mL	0.9567 mL	1.9134 mL	4.7836 mL
	25 mM	0.1531 mL	0.7654 mL	1.5307 mL	3.8269 mL
	30 mM	0.1276 mL	0.6378 mL	1.2756 mL	3.1891 mL
	40 mM	0.0957 mL	0.4784 mL	0.9567 mL	2.3918 mL
	50 mM	0.0765 mL	0.3827 mL	0.7654 mL	1.9134 mL
	60 mM	0.0638 mL	0.3189 mL	0.6378 mL	1.5945 mL
	80 mM	0.0478 mL	0.2392 mL	0.4784 mL	1.1959 mL
	100 mM	0.0383 mL	0.1913 mL	0.3827 mL	0.9567 mL

* Note: If you choose water as the stock solution, please dilute it to the working solution, then filter and sterilize it with a 0.22 μm filter before use.