Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease.
D000596:Amino Acids / Q000378:metabolism*; D002208:Caproates / Q000378:metabolism*; D002849:Chromatography, Gas; D004032:Diet; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D007651:Keto Acids / Q000097:blood / Q000302:isolation & purification* / Q000652:urine; D008375:Maple Syrup Urine Disease / Q000188:drug therapy / Q000378:metabolism*; D010091:Oximes; D000439:Pentanols / Q000378:metabolism*; D010641:Phenotype; D011768:Pyruvate Dehydrogenase Complex / Q000037:antagonists & inhibitors; D013831:Thiamine / Q000008:administration & dosage; D014631:Valerates / Q000378:metabolism*
D000596:Amino Acids; D002208:Caproates; D005227:Fatty Acids; D007651:Keto Acids; D010091:Oximes; D000439:Pentanols; D011768:Pyruvate Dehydrogenase Complex; D014631:Valerates; D013831:Thiamine