The role of the blood-brain barrier in the aetiology of permanent brain dysfunction in hyperphenylalaninaemia

Journal of Inherited Metabolic Disease
F Hommes

Abstract

Calculations on the rate of entry of the neutral amino acids into the brain via the blood-brain barrier show that a considerable decrease in this rate, particularly for tryptophan and tyrosine, takes place in histidinaemia and tyrosinaemia, type II. These conditions are, however, not associated with mental retardation. It is therefore concluded that effects at the blood-brain barrier alone do not provide an adequate explanation for the aetiology of permanent brain dysfunction in hyperphenylalaninaemia.

References

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Citations

Feb 1, 1992·Amino Acids·M Hjelm, J Seakins
Jan 1, 1990·Journal of Inherited Metabolic Disease·F A Hommes, J S Lee
Jan 1, 1991·Journal of Inherited Metabolic Disease·A G AntoshechkinG P Railian
Jan 1, 1995·Journal of Inherited Metabolic Disease·G M KnudsenH Lou
Sep 22, 2006·Journal of Inherited Metabolic Disease·R MatalonF Guttler
Oct 1, 1994·Journal of Clinical and Experimental Neuropsychology·E SchmidtL de Sonneville
Jan 17, 2004·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Reuben MatalonFlemming Guttler
Dec 1, 1991·Biochemical Medicine and Metabolic Biology·F A Hommes
Nov 11, 1998·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·H E MöllerK Ullrich
Dec 16, 2000·Journal of Inherited Metabolic Disease·C J WilsonP T Clayton
Jul 13, 2011·Developmental Medicine and Child Neurology·Corinne De LaetPhilippe J Goyens

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