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3-Hydroxyisovalerylcarnitine

Product Specifications

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

Product Datasheet

https://www.targetmol.com/attachment/DataSheet/212215416682971166/TN7605

CAS Number

99159-87-2

Available Sizes

Curated Selection

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