3-Hydroxyisovalerylcarnitine

MedChem Express

3-Hydroxyisovalerylcarnitine [99159-87-2]

5

99159-87-2

Research Use Only

99.40

C12H23NO5

261.31

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] .

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

-20°C

12352200