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Fiche 1,  Justifications,  Anglais

Record number: 1, Textual support number: 1 CONT

Maple syrup urine disease (MSUD). This disorder, also called branched-chain a-ketoaciduria, derives its name from the burnt-sugar smell of affected infants. MSUD is caused by impaired branched chain a-ketoacid dehydrogenase, which catalyzes decarboxylation of the a-ketoacid derivatives of all three of these amino acids.  1, fiche 1, Anglais, - maple%20syrup%20urine%20disease

Fiche 1,  Justifications,  Français

Record number: 1, Textual support number: 1 DEF

Maladie héréditaire à transmission autosomique récessive, liée à un déficit de l'enzyme catalysant la décarboxylation oxydative des cétoacides dérivant des acides ramifiés (leucine, isoleucine et valine).  3, fiche 1, Français, - maladie%20des%20urines%20%C3%A0%20odeur%20de%20sirop%20d%27%C3%A9rable

Fiche 1,  Justifications,  Espagnol