3-Hydroxyisovalerylcarnitine (Standard) [CAS 99159-87-2]
3-Hydroxyisovalerylcarnitine (Standard) [CAS 99159-87-2]
SKU:QM-0021773
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Product Details
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Description
3-Hydroxyisovalerylcarnitine (Standard) is the analytical standard of 3-Hydroxyisovalerylcarnitine. This product is intended for research and analytical applications. 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].
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CAS Number
[99159-87-2]
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Molecular Formula
C12H23NO5
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Molecular Weight
261.31
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Smiles
CC(O)(CC(O[C@@H](C[N+](C)(C)C)CC([O-])=O)=O)C
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Target
Endogenous Metabolite; Reference Standards
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Shipping Conditions
Room temperature
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3-Hydroxyisovalerylcarnitine (Standard) [CAS 99159-87-2]
3-Hydroxyisovalerylcarnitine (Standard) is a premium-grade research compound supplied by Quantimol for laboratory and scientific applications.