title: [Maple syrup urine disease: molecular pathology of the branched chain alpha-keto acid dehydrogenase complex].
authors.affiliation: Department of Pediatrics, Kumamoto University Medical School.
authors.forename: Y
authors.initials: Y
authors.lastname: Nobukuni
authors.forename: H
authors.initials: H
authors.lastname: Mitsubuchi
authors.forename: Y
authors.initials: Y
authors.lastname: Indo
authors.forename: F
authors.initials: F
authors.lastname: Endo
authors.forename: I
authors.initials: I
authors.lastname: Matsuda
mesh_terms: D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); D000818:Animals; D001483:Base Sequence; D002874:Chromosome Mapping; D003001:Cloning, Molecular; D004247:DNA; D006801:Humans; D007658:Ketone Oxidoreductases / Q000235:genetics* / Q000378:metabolism / Q000502:physiology; D008375:Maple Syrup Urine Disease / Q000201:enzymology / Q000235:genetics*; D008928:Mitochondria / Q000201:enzymology; D008969:Molecular Sequence Data; D009097:Multienzyme Complexes / Q000235:genetics* / Q000378:metabolism / Q000502:physiology; D009154:Mutation; D010766:Phosphorylation
chemical_list: D009097:Multienzyme Complexes; D004247:DNA; D007658:Ketone Oxidoreductases; D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)