Classical maple syrup urine disease: cofactor resistance.
D000596:Amino Acids / Q000652:urine; D002247:Carbon Isotopes; D002478:Cells, Cultured; D003065:Coenzyme A / Q000494:pharmacology; D003067:Coenzymes / Q000494:pharmacology*; D005260:Female; D005347:Fibroblasts / Q000187:drug effects / Q000378:metabolism*; D005182:Flavin-Adenine Dinucleotide / Q000494:pharmacology; D005826:Genetics, Medical; D006801:Humans; D007231:Infant, Newborn; D007532:Isoleucine / Q000378:metabolism; D007651:Keto Acids / Q000652:urine; D007930:Leucine / Q000378:metabolism; D008297:Male; D008375:Maple Syrup Urine Disease / Q000201:enzymology / Q000378:metabolism*; D009243:NAD / Q000494:pharmacology; D011732:Pyridoxal Phosphate / Q000494:pharmacology; D011773:Pyruvates / Q000378:metabolism; D012867:Skin; D013835:Thiamine Pyrophosphate / Q000494:pharmacology; D008063:Thioctic Acid / Q000494:pharmacology; D014633:Valine / Q000378:metabolism
D000596:Amino Acids; D002247:Carbon Isotopes; D003067:Coenzymes; D007651:Keto Acids; D011773:Pyruvates; D007532:Isoleucine; D009243:NAD; D005182:Flavin-Adenine Dinucleotide; D011732:Pyridoxal Phosphate; D008063:Thioctic Acid; D007930:Leucine; D014633:Valine; D013835:Thiamine Pyrophosphate; D003065:Coenzyme A