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Metabolism of BCAAs

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Branched Chain Amino Acids in Clinical Nutrition

Part of the book series: Nutrition and Health ((NH))

Abstract

The three branched chain amino acids are unique in that the first catabolic step does not occur in the liver. Leucine, isoleucine, and valine share the first two catabolic enzymes—branched chain aminotransferase (BCAT) and branched chain ketoacid dehydrogenase (BCKD). Both BCAT isoforms use Vitamin B-6 cofactors as temporary acceptors of the α-amino group during the process of aminating α-ketoglutarate, which becomes glutamate. The deaminated BCAAs are known as BCKAs, and can be reaminated into their BCAA form. The next step in BCAA catabolism, the decarboxylation of the BCKAs, is irreversible. In some tissues, the mitochondrial isoform of BCAT (BCATm), appears to form a metabolon with BCKD, which is a physical relationship between the BCATm enzyme and the BCKD enzyme complex. This facilitates the transfer of substrates and increases the efficiency of the reactions. After decarboxylation of the BCKA, the catabolic pathways for the three BCAA precursors diverge, utilizing different enzymes and processes. Disruptions in BCAA metabolism, primarily occurring in the first two steps, can cause a number of severe metabolic disorders. Additionally, changes in both BCAAs and BCAA enzymes have been implicated in the pathophysiology of TBI.

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Authors and Affiliations

  1. Department of Neurology, Uniformed Services University of the Health Sciences, Bethesda, MD, USA

    Jeffrey T. Cole Ph.D., M.S.

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  1. Jeffrey T. Cole Ph.D., M.S.
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Correspondence to Jeffrey T. Cole Ph.D., M.S. .

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Editors and Affiliations

  1. Intensive Care, Barnet and Chase Farm Hospitals, Royal Free London NHS Foundation Trust, London, United Kingdom

    Rajkumar Rajendram

  2. Diabetes and Nutritional Sciences, Kings College London, London, United Kingdom

    Victor R. Preedy

  3. Department of Biomedical Sciences, University of Westminster, London, United Kingdom

    Vinood B. Patel

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Cole, J.T. (2015). Metabolism of BCAAs. In: Rajendram, R., Preedy, V., Patel, V. (eds) Branched Chain Amino Acids in Clinical Nutrition. Nutrition and Health. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-1923-9_2

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  • DOI: https://doi.org/10.1007/978-1-4939-1923-9_2

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  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4939-1922-2

  • Online ISBN: 978-1-4939-1923-9

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