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].
Molecular Weight:
261.31
Purity:
99.40
CAS Number:
[99159-87-2]
Formula:
C12H23NO5
Target:
Endogenous Metabolite
Application Notes:
MCE Product type: Natural Products
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