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Literature DB >> 2501582

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

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.

Entities: Chemical Disease

Mesh：

Substances：
Amino Acids
Phenylalanine

Year: 1989 PMID： 2501582 DOI： 10.1007/bf01805529

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

27 in total

1. [On the pathogenesis of feeble-mindedness in phenylketonuria].

Authors: F LINNEWEH; M EHRLICH
Journal: Klin Wochenschr Date: 1962-03-01

2. Where do the depleted plasma amino acids go in phenylketonuria?

Authors: H N Christensen
Journal: Biochem J Date: 1986-06-15 Impact factor: 3.857

3. Large doses of tryptophan and tyrosine as potential therapeutic alternative to dietary phenylalanine restriction in phenylketonuria.

Authors: H Lou
Journal: Lancet Date: 1985-07-20 Impact factor: 79.321

Review 4. Brain metabolism: a perspective from the blood-brain barrier.

Authors: W M Pardridge
Journal: Physiol Rev Date: 1983-10 Impact factor: 37.312

5. Brain protein and myelin metabolism in hyperphenylalaninemic rats.

Authors: R Berger; J Springer; F A Hommes
Journal: Cell Mol Biol Incl Cyto Enzymol Date: 1980

6. Follow-up study of a nation-wide neonatal metabolic screening program in Japan. A collaborative study group of neonatal screening for inborn errors of metabolism in Japan.

Authors: K Tada; H Tateda; S Arashima; K Sakai; T Kitagawa; K Aoki; S Suwa; M Kawamura; T Oura; M Takesada
Journal: Eur J Pediatr Date: 1984-08 Impact factor: 3.183

9. The effect of plasma valine, isoleucine and leucine on the control of the flux through tyrosine- and tryptophan-hydroxylase in the brain.

Authors: F A Hommes; J S Lee
Journal: J Inherit Metab Dis Date: 1990 Impact factor: 4.982

10. State regulation and response inhibition in children with ADHD and children with early- and continuously treated phenylketonuria: an event-related potential comparison.

Authors: J R Wiersema; J J van der Meere; H Roeyers
Journal: J Inherit Metab Dis Date: 2005 Impact factor: 4.750

10 in total

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

1. [On the pathogenesis of feeble-mindedness in phenylketonuria].

2. Where do the depleted plasma amino acids go in phenylketonuria?

3. Large doses of tryptophan and tyrosine as potential therapeutic alternative to dietary phenylalanine restriction in phenylketonuria.

Review 4. Brain metabolism: a perspective from the blood-brain barrier.

5. Brain protein and myelin metabolism in hyperphenylalaninemic rats.

6. Follow-up study of a nation-wide neonatal metabolic screening program in Japan. A collaborative study group of neonatal screening for inborn errors of metabolism in Japan.

7. Reduction of cerebrospinal fluid phenylalanine after oral administration of valine, isoleucine, and leucine.

8. A new approach to the treatment of phenylketonuria.

Review 9. Neutral amino acid transport at the human blood-brain barrier.

Review 10. Progress in experimental phenylketonuria: a critical review.

1. Modelling amino acid metabolism.

2. The control of 5-hydroxytryptamine and dopamine synthesis in the brain: a theoretical approach.

3. Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria.

4. Large neutral amino acids in the treatment of phenylketonuria (PKU).

5. Phenylalanine supplementation improves the phenylalanine profile in tyrosinaemia.

6. Blood-brain barrier transport of amino acids in healthy controls and in patients with phenylketonuria.

7. Phenylketonuria: an inborn error of phenylalanine metabolism.

8. Content of phenylalanine, tyrosine and their metabolites in CSF in phenylketonuria.

9. The effect of plasma valine, isoleucine and leucine on the control of the flux through tyrosine- and tryptophan-hydroxylase in the brain.

10. State regulation and response inhibition in children with ADHD and children with early- and continuously treated phenylketonuria: an event-related potential comparison.