The enzyme defect in maple syrup urine disease (branched chain ketoaciduria).
D000818:Animals; D002262:Carboxy-Lyases / Q000378:metabolism; D002675:Child, Preschool; D004035:Diet Therapy; D005260:Female; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D007651:Keto Acids / Q000378:metabolism*; D007962:Leukocytes / Q000201:enzymology; D008099:Liver / Q000201:enzymology; D008297:Male; D008375:Maple Syrup Urine Disease / Q000201:enzymology*; D051381:Rats; D012867:Skin / Q000473:pathology
D007651:Keto Acids; D002262:Carboxy-Lyases