3-Hydroxyisovalerylcarnitine in patients with deficiency of 3-methylcrotonyl CoA carboxylase

Clin Chim Acta. 1995 Aug 31;240(1):35-51. doi: 10.1016/0009-8981(95)06126-2.
No abstract available

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Carbon-Carbon Ligases*
  • Carnitine / analogs & derivatives*
  • Carnitine / therapeutic use
  • Carnitine / urine
  • Child, Preschool
  • Female
  • Gas Chromatography-Mass Spectrometry
  • Glycine / analogs & derivatives
  • Glycine / urine
  • Humans
  • Ligases / deficiency*
  • Male
  • Valerates / urine

Substances

  • 3-hydroxyisovalerylcarnitine
  • Valerates
  • beta-methylcrotonylglycine
  • beta-hydroxyisovaleric acid
  • Ligases
  • Carbon-Carbon Ligases
  • methylcrotonoyl-CoA carboxylase
  • Carnitine
  • Glycine