Significant phenylalanine hydroxylation in vivo in patients with classical phenylketonuria (Q34256852)

31 July 2017

title

Significant phenylalanine hydroxylation in vivo in patients with classical phenylketonuria (English)

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31 July 2017

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Thompson GN

1

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31 July 2017

Halliday D

series ordinal

2

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31 July 2017

language of work or name

English

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publication date

1 July 1990

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Journal of Clinical Investigation

31 July 2017

published in

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31 July 2017

volume

86

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31 July 2017

issue

1

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31 July 2017

page(s)

317-322

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Whole-body leucine and lysine metabolism: response to dietary protein intake in young men.

31 July 2017

cites work

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Detection of heterozygous carriers for phenylketonuria by a L-[2H5]phenylalanine stable isotope loading test.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

In vivo safety of hollow fiber enzyme-reactors with immobilized phenylalanine ammonia-lyase in a large animal model for phenylketonuria

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Tracers in metabolic research: radioisotope and stable isotope/mass spectrometry methods.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Muscle Protein Synthesis Measured by Stable Isotope Techniques in Man: The Effects of Feeding and Fasting

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Quantitative studies on the urinary excretion of unconjugated aromatic acids in phenylketonuria.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Detection of hepatic phenylalanine 4-hydroxylase in classical phenylketonuria

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Fasting Plasma Amino Acid Concentrations in PKU Children on Two Different Levels of Protein Intake

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Effects of phenylalanine loading on protein synthesis in the fetal heart and brain of rat: an experimental approach to maternal phenylketonuria

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Heterozygote detection in phenylketonuria. Measurement of discriminatory ability and interpretation of the phenylalanine loading test by determination of the heterozygote likelihood ratio

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Classic phenylketonuria: Diagnosis through heterozygote detection

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Detection of heterozygotes for phenylketonuria. Total body phenylalanine clearance and concentrations of phenylalanine and tyrosine in the plasms of fasting subjects compared

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

In vivo studies of the phenylalanine-4-hydroxylase system in hyperphenylalaninemics and phenylketonurics

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

The labelling of urinary acids after oral doses of deuterated l-phenylalanine and l-tyrosine in normal subjects. quantitative studies with implications for the deuterated phenylalanine load test in phenylketonuria

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Sensitive in vivo assay of the phenylalanine hydroxylating system with a small intravenous dose of heptadeutero l-phenylalanine using high pressure liquid chromatography and capillary gas chromatography/mass fragmentography

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Discrimination of phenylketonurics from persistent hyperphenylalaninemia patients using a simple phenylalanine loading test.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

[Classification and heterogeneity of hyperphenylalaninemias linked to a phenylalanine hydroxylase deficiency]

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=296723

Tolerance of Phenylalanine after Intravenous Administration in Phenylketonurics, Heterozygous Carriers, and Normal Adults

28 October 2018

1 reference

12 December 2020

Urinary amino acid excretion in phenylketonuric, hyperphenylalaninemic, and normal patients

1 reference

12 December 2020

Solubilization and partial purification of tyrosine hydroxylase from bovine adrenal medulla

1 reference

12 December 2020

Phenylketonuria: reduction of serum levels of phenylalanine following oral administration of B-2 thienylalanine

1 reference

12 December 2020

Phenylalanine Hydroxylase Activity in Liver Biopsies from Hyperphenylalaninemia Heterozygotes: Deviation from Proportionality with Gene Dosage

1 reference

12 December 2020

Determination of Phenylalanine Hydroxylase Activity in Patients with Phenylketonuria and Hyperphenylalaninemia

1 reference

12 December 2020

Determination of deuterium-labeled phenylalanine and tyrosine in human plasma with high pressure liquid chromatography and mass spectrometry

1 reference

12 December 2020

Substrate activation of phenylalanine hydroxylase. A kinetic characterization

1 reference

12 December 2020

The conversion of phenylalanine to tyrosine in man. Direct measurement by continuous intravenous tracer infusions of L-[ring-2H5]phenylalanine and L-[1-13C] tyrosine in the postabsorptive state

1 reference

12 December 2020

Relationship of plasma leucine and alpha-ketoisocaproate during a L-[1-13C]leucine infusion in man: a method for measuring human intracellular leucine tracer enrichment

1 reference

12 December 2020

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

1 reference

12 December 2020

The use of deuterated phenylalanine for the in vivo assay of phenylalanine hydroxylase activity in children

1 reference

12 December 2020

Comparative diagnostic value of phenylalanine challenge and phenylalanine hydroxylase activity in phenylketonuria

1 reference

12 December 2020

Oral versus intravenous L-phenylalanine loading compared by simultaneous application of L-[2H5] and L-[15N]phenylalanine

1 reference

12 December 2020

Relationships between plasma isotope enrichments of leucine and alpha-ketoisocaproic acid during continuous infusion of labelled leucine

1 reference

12 December 2020

Rapid measurement of whole body and forearm protein turnover using a [2H5]phenylalanine model

1 reference

12 December 2020

Detection of Heterozygotes for Phenylketonuria by Column Chromatography and Discriminatory Analysis

1 reference

12 December 2020

Identifiers

DOI

10.1172/JCI114701

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31 July 2017

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31 July 2017

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