(Q37384807)

20 August 2017

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title

Brain dysfunction in phenylketonuria: is phenylalanine toxicity the only possible cause? (English)

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20 August 2017

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F J van Spronsen

1

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PubMed ID

19191004

20 August 2017

Marieke Hoeksma

series ordinal

2

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PubMed ID

19191004

20 August 2017

Dirk-Jan Reijngoud

series ordinal

3

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20 August 2017

13 January 2009

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Journal of Inherited Metabolic Disease

20 August 2017

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20 August 2017

32

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20 August 2017

1

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20 August 2017

46-51

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https://scigraph.springernature.com/pub.10.1007/s10545-008-0946-2

20 August 2017

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cites work

Treatable neurotransmitter deficiency in mild phenylketonuria

1 reference

Crossref

https://api.crossref.org/works/10.1007%2FS10545-008-0946-2

Dietary problems of phenylketonuria: effect on CNS transmitters and their possible role in behaviour and neuropsychological function

21 January 2018

1 reference

12 December 2020

Tyrosine, phenylalanine, and catecholamine synthesis and function in the brain

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12 December 2020

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

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12 December 2020

Long-term changes in glutamatergic synaptic transmission in phenylketonuria

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12 December 2020

Evidence for central nervous system glial cell plasticity in phenylketonuria.

1 reference

12 December 2020

Motor function under lower and higher controlled processing demands in early and continuously treated phenylketonuria.

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12 December 2020

Cognitive deficits in a genetic mouse model of the most common biochemical cause of human mental retardation.

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12 December 2020

Executive function impairment in early-treated PKU subjects with normal mental development.

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12 December 2020

Is there a relationship between 3-hydroxy-3-methylglutaryl coenzyme a reductase activity and forebrain pathology in the PKU mouse?

1 reference

12 December 2020

Phenylalanine reduces synaptic density in mixed cortical cultures from mice.

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12 December 2020

Measurement of neurotransmitter metabolites in the cerebrospinal fluid of phenylketonuric patients under dietary treatment

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12 December 2020

Cerebral energy metabolism in phenylketonuria: findings by quantitative In vivo 31P MR spectroscopy

1 reference

12 December 2020

Neurotransmitter transporters and their impact on the development of psychopharmacology

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12 December 2020

Amino acid imbalance and intracellular protein synthesis

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12 December 2020

Executive functioning, memory, and learning in phenylketonuria.

1 reference

12 December 2020

Neuropsychology of early-treated phenylketonuria: specific executive function deficits

1 reference

12 December 2020

Behaviour and school achievement in patients with early and continuously treated phenylketonuria.

1 reference

12 December 2020

The neuropsychological profile of early and continuously treated phenylketonuria: orienting, vigilance, and maintenance versus manipulation-functions of working memory

1 reference

12 December 2020

Deficits in brain serotonin synthesis in a genetic mouse model of phenylketonuria

1 reference

12 December 2020

Effect of metals and phenylalanine on the activity of human tryptophan hydroxylase-2: comparison with that on tyrosine hydroxylase activity.

1 reference

12 December 2020

Monogenic traits are not simple: lessons from phenylketonuria

1 reference

12 December 2020

The behavioral profile of severe mental retardation in a genetic mouse model of phenylketonuria

1 reference

12 December 2020

Behaviour in early treated phenylketonuria: a systematic review

1 reference

12 December 2020

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

1 reference

12 December 2020

Individual blood-brain barrier phenylalanine transport determines clinical outcome in phenylketonuria

1 reference

12 December 2020

Cerebral protein synthesis in a genetic mouse model of phenylketonuria

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12 December 2020

A new approach to the treatment of phenylketonuria.

1 reference

12 December 2020

Relationship between myelin production and dopamine synthesis in the PKU mouse brain

1 reference

12 December 2020

The effects of phenylalanine on amino acid metabolism and protein synthesis in brain cells in vitro

1 reference

12 December 2020

Neurotransmitter defects and treatment of disorders of hyperphenylalaninemia

1 reference

12 December 2020

Are neuropsychological impairments in children with early-treated phenylketonuria (PKU) related to white matter abnormalities or elevated phenylalanine levels?

1 reference

12 December 2020

Executive functioning and speed of processing in phenylketonuria

1 reference

12 December 2020

The effects of large neutral amino acid supplements in PKU: an MRS and neuropsychological study.

1 reference

12 December 2020

Effects of dietary management of phenylketonuria on long-term cognitive outcome

1 reference

12 December 2020

Pathogenesis of different clinical outcomes in spite of identical genotypes and comparable blood phenylalanine concentrations in phenylketonurics.

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12 December 2020

Deficits in selective and sustained attention processes in early treated children with phenylketonuria--result of impaired frontal lobe functions?

1 reference

12 December 2020

Psychiatric disorders in adult patients with early-treated phenylketonuria.

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12 December 2020

Phenylketonuria: tyrosine supplementation in phenylalanine-restricted diets

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12 December 2020

Effects of concurrent phenylalanine levels on sustained attention and calculation speed in patients treated early for phenylketonuria.

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12 December 2020

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

1 reference

12 December 2020

Deficits in memory strategy use related to prefrontal dysfunction during early development: evidence from children with phenylketonuria

1 reference

12 December 2020

Rho proteins, mental retardation and the cellular basis of cognition

1 reference

12 December 2020

Solubilization and partial purification of tyrosine hydroxylase from bovine adrenal medulla

1 reference

12 December 2020

Brain plasticity in paediatric neurology

1 reference

12 December 2020

Dramatic brain aminergic deficit in a genetic mouse model of phenylketonuria

1 reference

12 December 2020

Effect of L-dopa on pattern visual evoked potentials (P-100) and neuropsychological tests in untreated adult patients with phenylketonuria

1 reference

12 December 2020

1 reference

12 December 2020

Impaired glutamatergic synaptic transmission in the PKU brain.

1 reference

12 December 2020

An animal model of early-treated PKU.

1 reference

12 December 2020

Blood-brain barrier phenylalanine transport and individual vulnerability in phenylketonuria.

1 reference

12 December 2020

The effects of high phenylalanine concentrations on serotinin and catecholamine metabolism in the human brain

1 reference

12 December 2020

Morphologic and histoanatomic observations of the brain in untreated human phenylketonuria.

1 reference

12 December 2020

Blood-brain barrier carrier-mediated transport and brain metabolism of amino acids

1 reference

12 December 2020

Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine

1 reference

12 December 2020

HYDROXYLATION OF PHENYLALANINE BY PURIFIED PREPARATIONS OF ADRENAL AND BRAIN TYROSINE HYDROXYLASE.

1 reference

12 December 2020

The neurochemistry of phenylketonuria.

1 reference

12 December 2020

10.1007/S10545-008-0946-2

Identifiers

DOI

1 reference

20 August 2017

1 reference

PubMed ID

19191004