3-Hydroxyisovalerylcarnitine - CAS 99159-87-2

3-Hydroxyisovalerylcarnitine is a carnitine derivative that may serve as a novel biomarker for biotin deficiency in humans, with its concentration increasing in such conditions. Biotin deficiency reduces the activity of biotin-dependent 3-methylcrotonyl-CoA carboxylase, hindering the conversion of 3-methylcrotonyl-CoA into 3-methylglutaconyl-CoA, and impairing the leucine catabolism pathway, resulting in elevated plasma 3-Hydroxyisovalerylcarnitine concentrations [1].

Endogenous Metabolite

Metabolism

99159-87-2

C12H23NO5

261.32

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

-20°C

Ice Packs

Natural Product