99159-87-2

3-Hydroxyisovalerylcarnitine Chemical Structure
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].

Cat. No. Product Name Purity Description Pricing
HY-N11200
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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HY-N11200R
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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