(Q42598809)

3 November 2017

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

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3 November 2017

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R Matalon

1

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3 November 2017

K Michals-Matalon

2

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3 November 2017

G Bhatia

3

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3 November 2017

E Grechanina

4

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3 November 2017

P Novikov

5

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3 November 2017

J D McDonald

6

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3 November 2017

J Grady

7

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3 November 2017

S K Tyring

8

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3 November 2017

F Guttler

9

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3 November 2017

21 September 2006

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Journal of Inherited Metabolic Disease

3 November 2017

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3 November 2017

29

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3 November 2017

6

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3 November 2017

732-738

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https://scigraph.springernature.com/pub.10.1007/s10545-006-0395-8

3 November 2017

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cites work

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

1 reference

Crossref

https://api.crossref.org/works/10.1007%2FS10545-006-0395-8

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

21 January 2018

1 reference

Crossref

https://api.crossref.org/works/10.1007%2FS10545-006-0395-8

Neurological deterioration in young adults with phenylketonuria

21 January 2018

1 reference

12 December 2020

Effect of age at loss of dietary control on intellectual performance and behavior of children with phenylketonuria

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12 December 2020

Tetrahydrobiopterin responsiveness in a large series of phenylketonuria patients

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12 December 2020

Characterization of mutations at the mouse phenylalanine hydroxylase locus

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12 December 2020

Influence of phenylalanine intake on phenylketonuria

1 reference

12 December 2020

Tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency: possible regulation of gene expression in a patient with the homozygous L48S mutation

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12 December 2020

Blood phenylalanine levels and intelligence of 10-year-old children with PKU in the National Collaborative Study

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12 December 2020

Neurological outcome in adult patients with early-treated phenylketonuria.

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12 December 2020

Biopterin responsive phenylalanine hydroxylase deficiency

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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

Cerebral protein synthesis in a genetic mouse model of phenylketonuria

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12 December 2020

Brain phenylalanine concentration in the management of adults with phenylketonuria

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12 December 2020

Return to diet therapy in patients with phenylketonuria

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12 December 2020

Tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency

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12 December 2020

Amino acid assignment to one of three blood-brain barrier amino acid carriers

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12 December 2020

Future role of large neutral amino acids in transport of phenylalanine into the brain

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12 December 2020

Phenylketonuric patients decades after diet.

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12 December 2020

Effect on intelligence of relaxing the low phenylalanine diet in phenylketonuria

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12 December 2020

How practical are recommendations for dietary control in phenylketonuria?

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12 December 2020

Tetrahydrobiopterin responsiveness in phenylketonuria differs between patients with the same genotype

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12 December 2020

New approaches to treat PKU: how far are we?

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12 December 2020

Kinetics of competitive inhibition of neutral amino acid transport across the blood-brain barrier.

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12 December 2020

Successful treatment of phenylketonuria with tetrahydrobiopterin

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12 December 2020

Brain MRI changes in phenylketonuria. Associations with dietary status.

1 reference

12 December 2020

Loss of intellectual function in children with phenylketonuria after relaxation of dietary phenylalanine restriction.

1 reference

12 December 2020

Tetrahydrobiopterin responsiveness in phenylketonuria. Two new cases and a review of molecular genetic findings

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12 December 2020

A treatment program for adolescents with phenylketonuria.

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12 December 2020

Transport of a large neutral amino acid (phenylalanine) in a human intestinal epithelial cell line: Caco-2.

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12 December 2020

Phenylketonuria: current dietary treatment practices in the United States and Canada

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12 December 2020

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

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12 December 2020

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

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

Tetrahydrobiopterin as an alternative treatment for mild phenylketonuria

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12 December 2020

Effect of stopping low-phenylalanine diet on intellectual progress of children with phenylketonuria

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12 December 2020

TRANSPORT OF NEUTRAL, DIBASIC AND N-METHYL-SUBSTITUTED AMINO ACIDS BY RAT INTESTINE

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12 December 2020

Intellectual development in 12-year-old children treated for phenylketonuria

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12 December 2020

Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents.

1 reference

12 December 2020

Sustained attention in adult phenylketonuria: the influence of the concurrent phenylalanine-blood-level

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12 December 2020

Tetrahydrobiopterin sensitivity in German patients with mild phenylalanine hydroxylase deficiency

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12 December 2020

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

1 reference

12 December 2020

Neuropsychologic functions of early treated patients with phenylketonuria, on and off diet: results of a cross-national and cross-sectional study.

1 reference

12 December 2020

Phenylalanine transport at the human blood-brain barrier. Studies with isolated human brain capillaries.

1 reference

12 December 2020

Neutral amino acid transport at the human blood-brain barrier

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12 December 2020

Kinetic analysis of blood-brain barrier transport of amino acids

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12 December 2020

Neuropsychological effects of subsequent exposure to phenylalanine in adolescents and young adults with early-treated phenylketonuria.

1 reference

12 December 2020

Early-treated phenylketonuria: adult neuropsychologic outcome.

1 reference

12 December 2020

Correction of kinetic and stability defects by tetrahydrobiopterin in phenylketonuria patients with certain phenylalanine hydroxylase mutations

1 reference

12 December 2020

10.1007/S10545-006-0395-8

Identifiers

DOI

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Dimensions Publication ID

3 November 2017

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