(Q24293412)

https://api.crossref.org/works/10.1074/JBC.M110.124016

sapropterin dihydrochloride

12 June 2024

main subject

cell biology

0 references

1 reference

phenylalanine hydroxylase

ChEBI release 2020-05-28

ChEBI ID

32120

1 reference

phenylalanine 4-monooxygenase activity

GOA release 2020-03-11

1 reference

GOA release 2020-03-11

1

Søren W. Gersting

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

12 June 2024

author name string

Michael Staudigl

2

0 references

Marietta S. Truger

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

12 June 2024

Dunja D. Messing

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

12 June 2024

Marta K. Danecka

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

12 June 2024

Christian P. Sommerhoff

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

12 June 2024

Kristina F. Kemter

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

12 June 2024

Ania C. Muntau

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

12 June 2024

language of work or name

English

0 references

publication date

1 October 2010

0 references

27 July 2010

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

full work available at URL

12 June 2024

http://www.jbc.org/article/S0021925819889603/pdf

file format

Portable Document Format

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20667834/pdf/?tool=EBI

12 June 2024

file format

Portable Document Format

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20667834/?tool=EBI

12 June 2024

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

http://intl.jbc.org/cgi/content/abstract/285/40/30686

12 June 2024

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

https://europepmc.org/articles/PMC2945563

12 June 2024

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

https://europepmc.org/articles/PMC2945563?pdf=render

12 June 2024

file format

Portable Document Format

content deliverer

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

https://doi.org/10.1074/jbc.m110.124016

12 June 2024

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20667834

https://api.elsevier.com/content/article/PII:S0021925819889603?httpAccept=text/xml

12 June 2024

1 reference

https://api.crossref.org/works/10.1074/JBC.M110.124016

https://api.elsevier.com/content/article/PII:S0021925819889603?httpAccept=text/plain

12 June 2024

1 reference

https://api.crossref.org/works/10.1074/JBC.M110.124016

https://syndication.highwire.org/content/doi/10.1074/jbc.M110.124016

12 June 2024

1 reference

https://api.crossref.org/works/10.1074/JBC.M110.124016

Journal of Biological Chemistry

12 June 2024

published in

0 references

volume

285

0 references

issue

40

0 references

page(s)

30686-97

0 references

cites work

A structural basis for the allosteric regulation of non-hydrolysing UDP-GlcNAc 2-epimerases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

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

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Crystal Structure of a Mammalian CTP: Phosphocholine Cytidylyltransferase Catalytic Domain Reveals Novel Active Site Residues within a Highly Conserved Nucleotidyltransferase Fold

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

2.0A resolution crystal structures of the ternary complexes of human phenylalanine hydroxylase catalytic domain with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine or L-norleucine...

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Crystal structure of the ternary complex of the catalytic domain of human phenylalanine hydroxylase with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine, and its implications for the mechanism of catalysis and substrate activation

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Crystal structure and site-specific mutagenesis of pterin-bound human phenylalanine hydroxylase

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

ON THE NATURE OF ALLOSTERIC TRANSITIONS: A PLAUSIBLE MODEL

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Synaptotagmin VI and VIII and syntaxin 2 are essential for the mouse sperm acrosome reaction

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Comparison of Experimental Binding Data and Theoretical Models in Proteins Containing Subunits*

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Efficacy of sapropterin dihydrochloride in increasing phenylalanine tolerance in children with phenylketonuria: a phase III, randomized, double-blind, placebo-controlled study

7 April 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Loss of function in phenylketonuria is caused by impaired molecular motions and conformational instability

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Flagellin glycosylation in Pseudomonas aeruginosa PAK requires the O-antigen biosynthesis enzyme WbpO

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Essential function of the built-in lid in the allosteric regulation of eukaryotic and archaeal chaperonins

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Kinetic and stability analysis of PKU mutations identified in BH4-responsive patients

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

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

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Phenylketonuria mutations in Europe

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

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

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Characterization of phenylketonuria missense substitutions, distant from the phenylalanine hydroxylase active site, illustrates a paradigm for mechanism and potential modulation of phenotype

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

The structural basis of the recognition of phenylalanine and pterin cofactors by phenylalanine hydroxylase: implications for the catalytic mechanism

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

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

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

The role of phenylalanine in structure-function relationships of phenylalanine hydroxylase revealed by radiation target analysis

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

The phenylalanine hydroxylating system.

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

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

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Co-operativity in monomeric enzymes

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Rapid determination of enzyme kinetics from fluorescence: overcoming the inner filter effect

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Mechanism of phenylalanine regulation of phenylalanine hydroxylase.

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

A simple purification of phenylalanine hydroxylase by substrate-induced hydrophobic chromatography

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Regulation of phenylalanine hydroxylase activity by phenylalanine in vivo, in vitro, and in perfused rat liver.

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Biochemical characterization of recombinant human phenylalanine hydroxylase produced in Escherichia coli.

30 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2945563

Tetrahydrobiopterin as an alternative treatment for mild phenylketonuria

19 June 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin, 6R-BH4) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomised placebo-controlled study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Probing the role of crystallographically defined/predicted hinge-bending regions in the substrate-induced global conformational transition and catalytic activation of human phenylalanine hydroxylase by single-site mutagenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Use of a fluorescence plate reader for measuring kinetic parameters with inner filter effect correction

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Spectroscopic and kinetic properties of unphosphorylated rat hepatic phenylalanine hydroxylase expressed in Escherichia coli. Comparison of resting and activated states.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Order of substrate binding in bacterial phenylalanine hydroxylase and its mechanistic implication for pterin-dependent oxygenases

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Mechanisms underlying responsiveness to tetrahydrobiopterin in mild phenylketonuria mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Kinetic analyses of the magnesium chelatase provide insights into the mechanism, structure, and formation of the complex

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Kinetics and thermodynamics of ligand binding by cytochrome P450 3A4.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Purification and characterization of a chimeric enzyme from Haemophilus influenzae Rd that exhibits glutathione-dependent peroxidase activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.124016

Substrate activation of phenylalanine hydroxylase. A kinetic characterization

21 January 2018

1 reference

12 December 2020

Separation and properties of the 6-diastereoisomers of l-erythro-tetrahydrobiopterin and their reactivities with phenylalanine hydroxylase

1 reference

12 December 2020

The stimulation of rat liver phenylalanine hydroxylase by lysolecithin and -chymotrypsin

1 reference

12 December 2020

An assay for picomole levels of tyrosine and related phenols and its application to the measurement of phenylalanine hydroxylase activity

1 reference

12 December 2020

Two components of a secreted cell number-counting factor bind to cells and have opposing effects on cAMP signal transduction in Dictyostelium

1 reference

12 December 2020

Regulation of rat liver phenylalanine hydroxylase. I. Kinetic properties of the enzyme's iron and enzyme reduction site

1 reference

12 December 2020

Regulation of rat liver phenylalanine hydroxylase. II. Substrate binding and the role of activation in the control of enzymatic activity

1 reference

12 December 2020

Regulation of rat liver phenylalanine hydroxylase. III. Control of catalysis by (6R)-tetrahydrobiopterin and phenylalanine

1 reference

12 December 2020

Tryptophan fluorescence of human phenylalanine hydroxylase produced in Escherichia coli

1 reference

12 December 2020

Sensitivity of CaM kinase II to the frequency of Ca2+ oscillations

1 reference

12 December 2020

Domain structure and stability of human phenylalanine hydroxylase inferred from infrared spectroscopy

1 reference

12 December 2020

A rigorous experimental framework for detecting protein oligomerization using bioluminescence resonance energy transfer

1 reference

12 December 2020

Assessment of tetrahydrobiopterin (BH4) responsiveness in phenylketonuria

1 reference

12 December 2020