Defective decarboxylase in branched chain ketoacid oxidase multienzyme complex in classic type of maple syrup urine disease.
D002262:Carboxy-Lyases / Q000378:metabolism*; D003653:Decarboxylation; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007651:Keto Acids; D007700:Kinetics; D008055:Lipids; D008375:Maple Syrup Urine Disease / Q000201:enzymology*; D008661:Metabolism, Inborn Errors; D008967:Molecular Biology; D010088:Oxidoreductases / Q000378:metabolism*; D014020:Tissue Extracts / Q000032:analysis; D014631:Valerates
D007651:Keto Acids; D008055:Lipids; D014020:Tissue Extracts; D014631:Valerates; D010088:Oxidoreductases; D002262:Carboxy-Lyases