title: Genetic defects in E3 component of alpha-keto acid dehydrogenase complexes.
authors.affiliation: Department of Biochemistry, School of Medicine and Biomedical Sciences, State University of New York, Buffalo 14214, USA.
authors.forename: M S
authors.initials: MS
authors.lastname: Patel
authors.forename: Y S
authors.initials: YS
authors.lastname: Hong
authors.forename: D S
authors.initials: DS
authors.lastname: Kerr
mesh_terms: D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); D001483:Base Sequence; D017931:DNA Primers; D006801:Humans; D007658:Ketone Oxidoreductases / Q000235:genetics* / Q000378:metabolism; D008375:Maple Syrup Urine Disease / Q000175:diagnosis / Q000235:genetics; D009097:Multienzyme Complexes / Q000235:genetics* / Q000378:metabolism
chemical_list: D017931:DNA Primers; D009097:Multienzyme Complexes; D007658:Ketone Oxidoreductases; D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)