Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria (Q24563321)

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A Rupp

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E Mayatepek

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D Rating

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C Boesch

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H J Bremer

Journal of Clinical Investigation

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language of work or name

English

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publication date

April 1999

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published in

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volume

103

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issue

8

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page(s)

1169-78

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cites work

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Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria. A model for the study of phenylalanine and brain function in man

27 September 2017

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

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Kinetic analysis of blood-brain barrier transport of amino acids

27 September 2017

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Valine, isoleucine, and leucine. A new treatment for phenylketonuria

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Neuropsychology of early-treated phenylketonuria: specific executive function deficits

27 September 2017

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Versatile frequency domain fitting using time domain models and prior knowledge.

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Phenylketonuria: findings at MR imaging and localized in vivo H-1 MR spectroscopy of the brain in patients with early treatment.

30 May 2018

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The effects of chronic hyperphenylalaninaemia on mouse brain protein synthesis can be prevented by other amino acids

30 May 2018

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Amino acid depletion in the blood and brain tissue of hyperphenylalaninemic rats is abolished by the administration of additional lysine: a contribution to the understanding of the metabolic defects in phenylketonuria.

30 May 2018

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Phenylalanine alters the mean power frequency of electroencephalograms and plasma L-dopa in treated patients with phenylketonuria.

30 May 2018

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30 May 2018

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Branched chain amino acids improve complex maze learning in rat offspring prenatally exposed to hyperphenylalaninemia: implications for maternal phenylketonuria.

30 May 2018

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Asymmetrical transport of amino acids across the blood-brain barrier in humans

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Kinetics of phenylalanine transport at the human blood-brain barrier investigated in vivo.

28 November 2018

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28 November 2018

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Inhibition by L-phenylalanine of tryptophan transport by synaptosomal plasma membrane vesicles: implications in the pathogenesis of phenylketonuria.

28 November 2018

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In vivo measurement of phenylalanine in human brain by proton nuclear magnetic resonance spectroscopy

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12 December 2020

Identification and quantitation of phenylalanine in the brain of patients with phenylketonuria by means of localized in vivo 1H magnetic-resonance spectroscopy

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12 December 2020

Inhibition of neutral amino acid transport across the human blood-brain barrier by phenylalanine

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12 December 2020

Nutritional value of essential amino acids in the treatment of adults with phenylketonuria

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12 December 2020

Utilisation of amino acid mixtures in adolescents with phenylketonuria

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12 December 2020

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

1 reference

12 December 2020

Recommendations on the dietary management of phenylketonuria. Report of Medical Research Council Working Party on Phenylketonuria

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12 December 2020

The dynamics of brain concentrations of phenylalanine and its clinical significance in patients with phenylketonuria determined by in vivo 1H magnetic resonance spectroscopy

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12 December 2020

Effect of high-dose tyrosine supplementation on brain function in adults with phenylketonuria

1 reference

12 December 2020

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

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12 December 2020

1H NMR studies of glucose transport in the human brain

1 reference

12 December 2020

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

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12 December 2020

Standardized loading test with protein for the differentiation of phenylketonuria from hyperphenylalaninaemia

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12 December 2020

release_jky6oj2kmrgufhdeywmac5z3le

Identifiers

DOI

10.1172/JCI5017

0 references

Fatcat ID

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/jky6oj2kmrgufhdeywmac5z3le

24 November 2022