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. 1969 Aug;63(4):1365–1369. doi: 10.1073/pnas.63.4.1365

INHIBITION OF HUMAN BRAIN PYRUVATE KINASE AND HEXOKINASE BY PHENYLALANINE AND PHENYLPYRUVATE: POSSIBLE RELEVANCE TO PHENYLKETONURIC BRAIN DAMAGE*

George Weber 1
PMCID: PMC223473  PMID: 5260939

Abstract

In phenylketonuria high levels of L-phenylalanine are present along with increased levels of phenylpyruvic acid. The present work shows that L-phenylalanine is a competitive inhibitor of human brain pyruvate kinase and phenylpyruvic acid is an inhibitor of human brain hexokinase. The enzymes have approximately the same Ki for these inhibitors in adult and in fetal human brain. However, in the fetal human brain the absolute activities for both enzymes are less than 10 per cent of those found in the adult. Thus, the fetal brain enzymes may be more vulnerable to inhibition by these compounds in the phenylketonurics. The inhibition of human brain pyruvate kinase and hexokinase by L-phenylalanine and phenylpyruvic acid may have a role in the brain damage in phenylketonurics.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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