Bioactivity | 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]. |
CAS | 99159-87-2 |
Formula | C12H23NO5 |
Molar Mass | 261.31 |
Appearance | 固体 |
Transport | 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) |
Reference | [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. |