Altered kinetic properties of the branched-chain alpha-keto acid dehydrogenase complex due to mutation of the beta-subunit of the branched-chain alpha-keto acid decarboxylase (E1) component in lymphoblastoid cells derived from patients with maple syrup urine disease.

Branched-chain alpha-keto acid dehydrogenase (BCKDH) complexes of lymphoblastoid cell lines derived from patients with classical maple syrup urine disease (MSUD) phenotypes were studied in terms of their catalytic functions and analyzed by immunoblotting, using affinity purified anti-bovine BCKDH an...

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Main Authors: Indo, Y, Kitano, A, Endo, F, Akaboshi, I, Matsuda, I
Formato: Artigo
Idioma:Inglês
Publicado em: 1987
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Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC442202/
https://ncbi.nlm.nih.gov/pubmed/3597778
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