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Structure, function and assembly of mammalian branched-chain α-ketoacid dehydrogenase complex

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Alpha-Keto Acid Dehydrogenase Complexes

Part of the book series: MCBU Molecular and Cell Biology Updates ((MCBU))

Abstract

The mammalian branched-chain α-ketoacid dehydrogenase complex (BCKDC) catalyzes the oxidative decarboxylation of the branched-chain α-ketoacids derived from valine, leucine, and isoleucine. The mammalian BCKDC is a member of the α-ketoacid dehydrogenase complexes comprising pyruvate dehydrogenase (PDC), α-ketoglutarate dehydrogenase (KGDC) and BCKDC with similar structure and function (Reed et al., 1985; Yeaman, 1989). All three of these complexes are located in the mitochondrial matrix compartment and are associated with the inner membrane. BCKDC is a highly assembled macromolecular structure with an estimated molecular mass of 3 to 4 million daltons. BCKDC isolated from bovine kidney and liver has been shown to contain three catalytic components (Pettit et al., 1978; Heffelfmger et al., 1983): a branched-chain α-ketoacid decarboxylase (E1), a dihydrolipoyl transacylase (E2) and a dihydrolipoyl dehydrogenase (E3). The E3 components are shared by PDC, KGDC and BCKDC (Yeaman, 1989). BCKDC also contains two regulatory enzymes, a specific kinase (Shimomura et al., 1990; Lee et al., 1991) and a specific phosphatase (Damuni et al., 1984), which modulate the activity of BCKDC by reversible phosphorylation-dephosphorylation.

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Authors and Affiliations

  1. Departments of Biochemistry, University of Texas Southwestern Medical Center, Dallas, TX, 75235, USA

    R. M. Wynn, J. R. Davie, M. Meng & D. T. Chuang

  2. Departments of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, 75235, USA

    R. M. Wynn

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  1. R. M. Wynn
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  2. J. R. Davie
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  3. M. Meng
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  4. D. T. Chuang
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Editors and Affiliations

  1. Department of Biochemistry School of Medicine and Biomedical Sciences, State University of New York, 140 Farber Hall 3435 Main Street, 14214, Buffalo, New York, USA

    Mulchand S. Patel

  2. Department of Biochemistry, Kansas State University, 104 Willard Hall, 66506, Manhattan, KS, USA

    Thomas E. Roche

  3. Department of Biochemistry and Molecular Biology, Indiana University School of Medicine, 635 Barnhill Drive, 46202, Indianapolis, IN, USA

    Robert A. Harris

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© 1996 Birkhäuser Verlag Basel/Switzerland

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Wynn, R.M., Davie, J.R., Meng, M., Chuang, D.T. (1996). Structure, function and assembly of mammalian branched-chain α-ketoacid dehydrogenase complex. In: Patel, M.S., Roche, T.E., Harris, R.A. (eds) Alpha-Keto Acid Dehydrogenase Complexes. MCBU Molecular and Cell Biology Updates. Birkhäuser Basel. https://doi.org/10.1007/978-3-0348-8981-0_7

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  • DOI: https://doi.org/10.1007/978-3-0348-8981-0_7

  • Publisher Name: Birkhäuser Basel

  • Print ISBN: 978-3-0348-9853-9

  • Online ISBN: 978-3-0348-8981-0

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