3-droxyisovalerylcarnitine is a carnitine derivative. 3-droxyisovaleryl 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-metlcrotonyl-CoA carboxylase, hinders the conversion of 3-metlcrotonyl-CoA into 3-metlglutaconyl-CoA, and impairs the leucine catabolism pathway; resulting in plasma 3-droxyisovalerylcarnitine concentrations rise.
References:
[1]. Horvath TD, et al. Quantitative measurement of plasma 3-droxyisovaleryl carnitine by LC-MS/MS as a novel biomarker of biotin status in humans. Anal Chem. 2010 May 15;82(10):4140-4.