3-Hydroxyisovalerylcarnitine
Product Specifications
CAS Number
99159-87-2
Target
Endogenous Metabolite
Related Pathways
Metabolism
Bioactivity
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].
Smiles
O([C@@H](C[N+](C)(C)C)CC([O-])=O)C(CC(C)(C)O)=O
Molecular Formula
C12H23NO5
Molecular Weight
261.32
Shipping Conditions
Cool pack
Storage Temperature
-20°C
Available Sizes
Curated Selection
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