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A nonsense mutation (R242X) in the branched-chain alpha-keto acid dehydrogenase E1alpha subunit gene (BCKDHA) as a cause of maple syrup urine disease. Mutations in brief no. 160. Online.

  • J. Chinsky
  • , M. Appel
  • , S. Almashanu
  • , P. Costeas
  • , N. Ambulos
  • , R. Carmi

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6 Scopus citations

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Biochemistry, Genetics and Molecular Biology