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3-Hydroxyisovalerylcarnitine (Standard)

CAT: 0804-HY-N11200RSize: 1 EachDry Ice: NoHazardous: No
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CAT#:0804-HY-N11200RSize:1 Each
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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].
CAS Number
99159-87-2
UNSPSC
12352211
Target
Endogenous Metabolite; Reference Standards
Related Pathways
Metabolic Enzyme/Protease; Others
Field of Research
Metabolic Disease
Smiles
CC(O)(CC(O[C@@H](C[N+](C)(C)C)CC([O-])=O)=O)C
Molecular Formula
C12H23NO5
Molecular Weight
261.31
References & Citations
[1]Horvath TD, et al. Quantitative measurement of plasma 3-hydroxyisovaleryl carnitine by LC-MS/MS as a novel biomarker of biotin status in humans. Anal Chem. 2010 May 15;82 (10) :4140-4.
Shipping Conditions
Room temperature
Scientific Category
Reference Standards

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