Dual Role of Transport Competition in Amino Acid Deprivation of the Central Nervous System by Hyperphenylalaninemia

  • Halvor N. Christensen
  • Carlos de Céspedes
Chapter

Abstract

Evidence is presented that an amino acid such as phenylalanine accumulating in the organism to excessive concentrations may occasion not only a direct inhibition of the passage of endogenous amino acids across the blood-brain barrier, but also muscle and liver sequestration of certain amino acids. These are not precisely the same amino acids whose passage to the brain undergoes direct inhibition. This action explains, we believe, the lowering of the plasma levels of the affected-amino acids and may further handicap the nutritional flow of amino acids to the brain. We propose that the total effect of these two actions determines which amino acids will be depleted in the brain in hyperphenylalaninemia (both experimental and phenyl-ketonuric) or in leucinemia or, possibly, other amino acid accumulations in the body.

Keywords

Amino Acid Transport Neutral Amino Acid Amino Acid Distribution Amino Acid Deprivation Bulky Side Chain 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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Copyright information

© Birkhäuser Boston 1988

Authors and Affiliations

  • Halvor N. Christensen
    • 1
  • Carlos de Céspedes
    • 1
  1. 1.Department of Biological ChemistryThe University of Michigan Medical SchoolAnn ArborUSA

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