99159-87-2
Chemical Structure
3-Hydroxyisovalerylcarnitine
- CAS No.: 99159-87-2
- Formula:C12H23NO5
- Molecular Weight:261.31
IUPAC Name: (R)-3-((3-hydroxy-3-methylbutanoyl)oxy)-4-(trimethylammonio)butanoate
InChIKey: IGLHHSKNBDXCEY-SECBINFHSA-N
SMILES: CC(O)(CC(O[C@@H](C[N+](C)(C)C)CC([O-])=O)=O)C
Biological Activity: 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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3-Hydroxyisovalerylcarnitine | 99.4% | 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. | ||||||||||||||||||||
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3-Hydroxyisovalerylcarnitine (Standard) | ≥98% | 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. | ||||||||||||||||||||
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