Oxidation of branched-chain amino acids (BCAAs) is tightly regulated in mammals. We review here the distribution and regulation of whole-body BCAA oxidation. Phosphorylation and dephosphorylation of the rate-limiting enzyme, branched-chain α-ketoacid dehydrogenase complex directly regulates BCAA oxidation, and various other indirect mechanisms of regulation also exist. Most tissues throughout the body are capable of BCAA oxidation, and the flux of oxidative BCAA disposal in each tissue is influenced by three key factors: 1. tissue-specific preference for BCAA oxidation relative to other fuels, 2. the overall oxidative activity of mitochondria within a tissue, and 3. total tissue mass. Perturbations in BCAA oxidation have been implicated in many disease contexts, underscoring the importance of BCAA homeostasis in overall health.
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February 2021
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In the brain, cocaine exposure results in mitochondrial DNA damage, depletion of ATP and increased oxidative stress, coupled with increased mitochondrial fission. Therapies preventing such bioenergetic impairment may hold promise in mitigating cocaine pathology and addiction. You can read more about this in the review by Thornton and colleagues (pp. 749–764) in this issue. Image provided by Claire Thornton.
Review Article|
February 24 2021
Whole-body metabolic fate of branched-chain amino acids
Megan C. Blair;
Megan C. Blair
1Cardiovascular Institute, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, U.S.A.
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Michael D. Neinast;
Michael D. Neinast
2Lewis-Sigler Institute for Integrative Genomics, Princeton University, Princeton, NJ, U.S.A.
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Zoltan Arany
1Cardiovascular Institute, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, U.S.A.
Correspondence: Zoltan Arany (zarany@pennmedicine.upenn.edu)
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Publisher: Portland Press Ltd
Received:
December 16 2020
Revision Received:
February 01 2021
Accepted:
February 03 2021
Online ISSN: 1470-8728
Print ISSN: 0264-6021
© 2021 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society
2021
Biochem J (2021) 478 (4): 765–776.
Article history
Received:
December 16 2020
Revision Received:
February 01 2021
Accepted:
February 03 2021
Citation
Megan C. Blair, Michael D. Neinast, Zoltan Arany; Whole-body metabolic fate of branched-chain amino acids. Biochem J 26 February 2021; 478 (4): 765–776. doi: https://doi.org/10.1042/BCJ20200686
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