(Q46528179)

English

The activity of wild-type and mutant phenylalanine hydroxylase and its regulation by phenylalanine and tetrahydrobiopterin at physiological and pathological concentrations: an isothermal titration calorimetry study

scientific article published on 3 June 2005

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scholarly article

1 reference

Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

The activity of wild-type and mutant phenylalanine hydroxylase and its regulation by phenylalanine and tetrahydrobiopterin at physiological and pathological concentrations: an isothermal titration calorimetry study (English)

1 reference

Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

1 reference

based on heuristic

inferred from title

1

1 reference

Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

Aurora Martinez

2

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Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

publication date

3 June 2005

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Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

Molecular Genetics and Metabolism

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Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

86 Suppl 1

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Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

S43-53

1 reference

Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

Partial characterization and three-dimensional-structural localization of eight mutations in exon 7 of the human phenylalanine hydroxylase gene associated with phenylketonuria.

1 reference

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In vitro expression analysis of mutations in phenylalanine hydroxylase: Linking genotype to phenotype and structure to function

1 reference

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7 January 2021

Expression analysis of phenylketonuria mutations. Effect on folding and stability of the phenylalanine hydroxylase protein

1 reference

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7 January 2021

Phenylketonuria: genotype-phenotype correlations based on expression analysis of structural and functional mutations in PAH.

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7 January 2021

PKU mutation (D143G) associated with an apparent high residual enzyme activity: expression of a kinetic variant form of phenylalanine hydroxylase in three different systems

1 reference

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7 January 2021

Mechanisms underlying responsiveness to tetrahydrobiopterin in mild phenylketonuria mutations

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Tetrahydrobiopterin as an alternative treatment for mild phenylketonuria

1 reference

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7 January 2021

Long-term treatment and diagnosis of tetrahydrobiopterin-responsive hyperphenylalaninemia with a mutant phenylalanine hydroxylase gene

1 reference

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7 January 2021

Efficiency of long-term tetrahydrobiopterin monotherapy in phenylketonuria

1 reference

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7 January 2021

The metabolic and molecular bases of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency

1 reference

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7 January 2021

Wild-type phenylalanine hydroxylase activity is enhanced by tetrahydrobiopterin supplementation in vivo: an implication for therapeutic basis of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency

1 reference

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7 January 2021

Tetrahydrobiopterin protects phenylalanine hydroxylase activity in vivo: implications for tetrahydrobiopterin-responsive hyperphenylalaninemia

1 reference

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7 January 2021

Pterin-Dependent Amino Acid Hydroxylases

1 reference

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7 January 2021

Activation of phenylalanine hydroxylase: effect of substitutions at Arg68 and Cys237.

1 reference

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Probing cofactor specificity in phenylalanine hydroxylase by molecular dynamics simulations

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Studies on the regulatory properties of the pterin cofactor and dopamine bound at the active site of human phenylalanine hydroxylase.

1 reference

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7 January 2021

Coordinate regulation of tetrahydrobiopterin turnover and phenylalanine hydroxylase activity in rat liver cells

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7 January 2021

Use of surface plasmon resonance for real-time measurements of the global conformational transition in human phenylalanine hydroxylase in response to substrate binding and catalytic activation

1 reference

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7 January 2021

L-phenylalanine binding and domain organization in human phenylalanine hydroxylase: a differential scanning calorimetry study.

1 reference

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7 January 2021

Substrate-induced conformational transition in human phenylalanine hydroxylase as studied by surface plasmon resonance analyses: the effect of terminal deletions, substrate analogues and phosphorylation

1 reference

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7 January 2021

Thermodynamic characterization of the binding of tetrahydropterins to phenylalanine hydroxylase

1 reference

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7 January 2021

Expression of recombinant human phenylalanine hydroxylase as fusion protein in Escherichia coli circumvents proteolytic degradation by host cell proteases. Isolation and characterization of the wild-type enzyme

1 reference

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7 January 2021

Enzyme kinetics determined using calorimetry: a general assay for enzyme activity?

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Steady-state kinetics of the glutaminase reaction of CTP synthase from Lactococcus lactis. The role of the allosteric activator GTP incoupling between glutamine hydrolysis and CTP synthesis.

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7 January 2021

Is substrate inhibition a consequence of allostery in aspartate transcarbamylase?

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7 January 2021

Structure/function relationships in human phenylalanine hydroxylase. Effect of terminal deletions on the oligomerization, activation and cooperativity of substrate binding to the enzyme.

1 reference

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7 January 2021

Isothermal titration calorimetry and differential scanning calorimetry as complementary tools to investigate the energetics of biomolecular recognition

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Structural parameterization of the binding enthalpy of small ligands

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Free amino acids in the caiman and rat

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Chromatographic analysis of pteridines

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7 January 2021

Biopterin cofactor biosynthesis: GTP cyclohydrolase, neopterin and biopterin in tissues and body fluids of mammalian species

1 reference

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7 January 2021

The fate of intravenously administered tetrahydrobiopterin and its implications for heterologous gene therapy of phenylketonuria

1 reference

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A comparison of kinetic and regulatory properties of the tetrameric and dimeric forms of wild-type and Thr427-->Pro mutant human phenylalanine hydroxylase: contribution of the flexible hinge region Asp425-Gln429 to the tetramerization and cooperativ

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7 January 2021

Identifiers

10.1016/J.YMGME.2005.04.008

1 reference

Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

1 reference

Europe PubMed Central

22 December 2017

http://europepmc.org/abstract/MED/15936235

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