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Cited by 53Amino Acid Enzymes and MetabolismBiochemical Acid Research StudiesMetabolism and Genetic Disorders

BCAA Metabolism and NH3 Homeostasis

Conway, M. E., Hutson, S. M.·2016·Advances in Neurobiology
DOI 10.1007/978-3-319-45096-4_5
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Abstract

The branched chain amino acids (BCAA) are essential amino acids required not only for growth and development, but also as nutrient signals and as nitrogen donors to neurotransmitter synthesis and glutamate/glutamine cycling. Transamination and oxidative decarboxylation of the BCAAs are catalysed by the branched-chain aminotransferase proteins (BCATm, mitochondrial and BCATc, cytosolic) and the branched-chain α-keto acid dehydrogenase enzyme complex (BCKDC), respectively. These proteins show tissue, cell compartmentation, and protein-protein interactions, which call for substrate shuttling or channelling and nitrogen transfer for oxidation to occur. Efficient regulation of these pathways is mediated through the redox environment and phosphorylation in response to dietary and hormonal stimuli. The wide distribution of these proteins allows for effective BCAA utilisation. We discuss how BCAT, BCKDC, and glutamate dehydrogenase operate in supramolecular complexes, allowing for efficient channelling of substrates. The role of BCAAs in brain metabolism is highlighted in rodent and human brain, where differential expression of BCATm indicates differences in nitrogen metabolism between species. Finally, we introduce a new role for BCAT, where a change in function is triggered by oxidation of its redox-active switch. Our understanding of how BCAA metabolism and nitrogen transfer is regulated is important as many studies now point to BCAA metabolic dysregulation in metabolic and neurod…

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  • BCAAs and other large neutral amino acids are transported readily across the blood-brain barrier for transamination or delivery to neurons.

    “BCAAs and other large neutral amino acids are transported quite readily across the BBB for transamination or delivery to neurons (Conway and Hutson, 2016;Oldendorf, 1971).”

    From The Microbiota-Gut-Brain Axis (2019) · cites this paper as Conway and Hutson, 2016

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Authors
Conway, M. E., & Hutson, S. M.
Affiliations
  • University of the West of England (GB)
  • Virginia Tech (US)
Corresponding author
Conway, M. E.
ORCID
Conway, M. E.
Venue
Advances in Neurobiology
Publisher
Springer International Publishing
Published
2016
Volume
13
Pages
99-132
ISSN
  • 2190-5215 (ISSN-L)
  • 2190-5223
DOI
10.1007/978-3-319-45096-4_5
OpenAlex
W2551950524
Cited by
53
References
7
Citations by year
2026: 3 · 2025: 7 · 2024: 9 · 2023: 3 · 2022: 7 · 2021: 4 · 2020: 6 · 2019: 2 · 2018: 4 · 2017: 7 · 2016: 1
FWCI
5.90
Type
book chapter
Language
en
Open access
closed
Licence
www.springer.com/tdm (unspecified)
Locations
  • Advances in neurobiology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Amino Acid Enzymes and Metabolism
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Biochemical Acid Research Studies
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Keywords
branched-chain amino acids, branched-chain amino acid metabolism, branched-chain amino acid aminotransferase, branched-chain ketoacid dehydrogenase, glutamate dehydrogenase, transamination, oxidative decarboxylation, substrate channeling, redox regulation, phosphorylation, brain metabolism, nitrogen transfer, neurodegenerative diseases, metabolic disorders
MeSH terms
Amino Acids, Branched-Chain, Ammonia, Animals, Brain, Homeostasis, Humans

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