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Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease.

AuthorsG Lancaster, O A Mamer, C R Scriver
JournalMetabolism: clinical and experimental (Metabolism) Vol. 23 Issue 3 Pg. 257-65 (Mar 1974) ISSN: 0026-0495 [Print] United States
PMID4813956 (Publication Type: Journal Article)
Chemical References
  • Amino Acids
  • Caproates
  • Fatty Acids
  • Keto Acids
  • Oximes
  • Pentanols
  • Pyruvate Dehydrogenase Complex
  • Valerates
  • Thiamine
Topics
  • Amino Acids (metabolism)
  • Caproates (metabolism)
  • Chromatography, Gas
  • Diet
  • Fatty Acids (metabolism)
  • Humans
  • Keto Acids (blood, isolation & purification, urine)
  • Maple Syrup Urine Disease (drug therapy, metabolism)
  • Oximes
  • Pentanols (metabolism)
  • Phenotype
  • Pyruvate Dehydrogenase Complex (antagonists & inhibitors)
  • Thiamine (administration & dosage)
  • Valerates (metabolism)

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