The role of thiamin in maple syrup urine disease

Ann N Y Acad Sci. 1982;378:404-21. doi: 10.1111/j.1749-6632.1982.tb31214.x.
No abstract available

Publication types

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

MeSH terms

  • 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
  • Amino Acids, Branched-Chain / blood
  • Cells, Cultured
  • Child
  • Child, Preschool
  • Dietary Proteins / administration & dosage
  • Drug Stability
  • Female
  • Fibroblasts / metabolism
  • Hot Temperature
  • Humans
  • Infant
  • Keto Acids / metabolism
  • Ketone Oxidoreductases / metabolism*
  • Kinetics
  • Lymphocytes / metabolism
  • Male
  • Maple Syrup Urine Disease / diet therapy
  • Maple Syrup Urine Disease / drug therapy
  • Maple Syrup Urine Disease / metabolism*
  • Mitochondria, Liver / enzymology
  • Multienzyme Complexes / metabolism*
  • Protein Conformation
  • Thiamine / therapeutic use*
  • Thiamine Pyrophosphate / metabolism
  • Thiamine Pyrophosphate / pharmacology*

Substances

  • Amino Acids, Branched-Chain
  • Dietary Proteins
  • Keto Acids
  • Multienzyme Complexes
  • Ketone Oxidoreductases
  • 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
  • Thiamine Pyrophosphate
  • Thiamine