3-Hydroxyisovalerylcarnitine (standaard)

3-Hydroxyisovalerylcarnitine (Standard) is the analytical standard of 3-Hydroxyisovalerylcarnitine. This product is intended for research and analytical applications. 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].

99159-87-2

12352211

Endogenous Metabolite; Reference Standards

Metabolic Enzyme/Protease; Others

Metabolic Disease

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

C12H23NO5

261.31

[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.

Room temperature

http://file.medchemexpress.com/batch_PDF/HY-N11200R/

Reference Standards