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

Authors :: Lancaster G
Mamer OA
Scriver CR
Source :: Metabolism: clinical and experimental [Metabolism] 1974 Mar; Vol. 23 (3), pp. 257-65.
Publication Year :: 1974

Subjects :: Chromatography, Gas
Diet
Humans
Keto Acids blood
Keto Acids urine
Maple Syrup Urine Disease drug therapy
Phenotype
Pyruvate Dehydrogenase Complex antagonists & inhibitors
Thiamine administration & dosage
Amino Acids metabolism
Caproates metabolism
Fatty Acids metabolism
Keto Acids isolation & purification
Maple Syrup Urine Disease metabolism
Oximes
Pentanols metabolism
Valerates metabolism

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