The role of thiamin in maple syrup urine disease.
D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); D000597:Amino Acids, Branched-Chain / Q000097:blood; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D004044:Dietary Proteins / Q000008:administration & dosage; D004355:Drug Stability; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D006358:Hot Temperature; D006801:Humans; D007223:Infant; D007651:Keto Acids / Q000378:metabolism; D007658:Ketone Oxidoreductases / Q000378:metabolism*; D007700:Kinetics; D008214:Lymphocytes / Q000378:metabolism; D008297:Male; D008375:Maple Syrup Urine Disease / Q000178:diet therapy / Q000188:drug therapy / Q000378:metabolism*; D008930:Mitochondria, Liver / Q000201:enzymology; D009097:Multienzyme Complexes / Q000378:metabolism*; D011487:Protein Conformation; D013831:Thiamine / Q000627:therapeutic use*; D013835:Thiamine Pyrophosphate / Q000378:metabolism / Q000494:pharmacology*
D000597:Amino Acids, Branched-Chain; D004044:Dietary Proteins; D007651:Keto Acids; D009097:Multienzyme Complexes; D007658:Ketone Oxidoreductases; D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); D013835:Thiamine Pyrophosphate; D013831:Thiamine