3-Hydroxyisovalerylcarnitine

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[1].

Product Specifications

CAS Number

[99159-87-2]

UNSPSC

12352211

Hazard Statement

H302, H315, H319, H335

Target

Endogenous Metabolite

Type

Natural Products

Related Pathways

Metabolic Enzyme/Protease

Applications

Metabolism-protein/nucleotide metabolism

Field of Research

Metabolic Disease

Assay Protocol

https://www.medchemexpress.com/3-hydroxyisovalerylcarnitine.html

Purity

99.40

Solubility

H2O : 1 mg/mL (ultrasonic; warming) |DMSO : 50 mg/mL

Smiles

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

Molecular Formula

C12H23NO5

Molecular Weight

261.31

Precautions

H302, H315, H319, H335

References & Citations

[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.|[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.