Literature DB >> 22005392

Allosteric regulation of phenylalanine hydroxylase.

Paul F Fitzpatrick1.   

Abstract

The liver enzyme phenylalanine hydroxylase is responsible for conversion of excess phenylalanine in the diet to tyrosine. Phenylalanine hydroxylase is activated by phenylalanine; this activation is inhibited by the physiological reducing substrate tetrahydrobiopterin. Phosphorylation of Ser16 lowers the concentration of phenylalanine for activation. This review discusses the present understanding of the molecular details of the allosteric regulation of the enzyme. Copyright Â
© 2011 Elsevier Inc. All rights reserved.

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Year:  2011        PMID： 22005392      PMCID： PMC3271142          DOI： 10.1016/j.abb.2011.09.012

Source DB:  PubMed          Journal:  Arch Biochem Biophys        ISSN： 0003-9861            Impact factor:   4.013


  99 in total

1.  Characterization of chimeric pterin-dependent hydroxylases: contributions of the regulatory domains of tyrosine and phenylalanine hydroxylase to substrate specificity.

Authors:  S C Daubner; P J Hillas; P F Fitzpatrick
Journal:  Biochemistry       Date:  1997-09-30       Impact factor: 3.162

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

Authors:  R Shiman; G E Mortimore; C M Schworer; D W Gray
Journal:  J Biol Chem       Date:  1982-10-10       Impact factor: 5.157

3.  Substrate activation of phenylalanine hydroxylase. A kinetic characterization.

Authors:  R Shiman; D W Gray
Journal:  J Biol Chem       Date:  1980-05-25       Impact factor: 5.157

4.  Rat liver phenylalanine hydroxylase. Activation by sulfhydryl modification.

Authors:  M A Parniak; S Kaufman
Journal:  J Biol Chem       Date:  1981-07-10       Impact factor: 5.157

5.  Direct evidence for a phenylalanine site in the regulatory domain of phenylalanine hydroxylase.

Authors:  Jun Li; Udayar Ilangovan; S Colette Daubner; Andrew P Hinck; Paul F Fitzpatrick
Journal:  Arch Biochem Biophys       Date:  2010-10-14       Impact factor: 4.013

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

Authors:  Therese Solstad; Anne J Stokka; Ole A Andersen; Torgeir Flatmark
Journal:  Eur J Biochem       Date:  2003-03

7.  Ligand effects on the phosphorylation state of hepatic phenylalanine hydroxylase.

Authors:  R S Phillips; S Kaufman
Journal:  J Biol Chem       Date:  1984-02-25       Impact factor: 5.157

8.  Glucagon stimulation of rat hepatic phenylalanine hydroxylase through phosphorylation in vivo.

Authors:  J Donlon; S Kaufman
Journal:  J Biol Chem       Date:  1978-10-10       Impact factor: 5.157

9.  Correlation of rat hepatic phenylalanine hydroxylase, with tetrahydrobiopterin and GTP concentrations.

Authors:  T Guerin; G A Walsh; J Donlon; S Kaufman
Journal:  Int J Biochem Cell Biol       Date:  1998-09       Impact factor: 5.085

10.  Phosphorylation and mutations of Ser(16) in human phenylalanine hydroxylase. Kinetic and structural effects.

Authors:  Frederico Faria Miranda; Knut Teigen; Matthías Thórólfsson; Randi M Svebak; Per M Knappskog; Torgeir Flatmark; Aurora Martínez
Journal:  J Biol Chem       Date:  2002-08-15       Impact factor: 5.157
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  31 in total

Review 1.  X-ray Scattering Studies of Protein Structural Dynamics.

Authors:  Steve P Meisburger; William C Thomas; Maxwell B Watkins; Nozomi Ando
Journal:  Chem Rev       Date:  2017-05-30       Impact factor: 60.622

2.  Kinetic mechanism of phenylalanine hydroxylase: intrinsic binding and rate constants from single-turnover experiments.

Authors:  Kenneth M Roberts; Jorge Alex Pavon; Paul F Fitzpatrick
Journal:  Biochemistry       Date:  2013-01-29       Impact factor: 3.162

3.  Impact of quaternary structure dynamics on allosteric drug discovery.

Authors:  Eileen K Jaffe
Journal:  Curr Top Med Chem       Date:  2013       Impact factor: 3.295

4.  The phenylketonuria-associated substitution R68S converts phenylalanine hydroxylase to a constitutively active enzyme but reduces its stability.

Authors:  Crystal A Khan; Steve P Meisburger; Nozomi Ando; Paul F Fitzpatrick
Journal:  J Biol Chem       Date:  2019-01-23       Impact factor: 5.157

5.  Structure of full-length human phenylalanine hydroxylase in complex with tetrahydrobiopterin.

Authors:  Marte Innselset Flydal; Martín Alcorlo-Pagés; Fredrik Gullaksen Johannessen; Siseth Martínez-Caballero; Lars Skjærven; Rafael Fernandez-Leiro; Aurora Martinez; Juan A Hermoso
Journal:  Proc Natl Acad Sci U S A       Date:  2019-05-22       Impact factor: 11.205

6.  Computational study of missense mutations in phenylalanine hydroxylase.

Authors:  Kamila Réblová; Petr Kulhánek; Lenka Fajkusová
Journal:  J Mol Model       Date:  2015-03-07       Impact factor: 1.810

7.  A new model for allosteric regulation of phenylalanine hydroxylase: implications for disease and therapeutics.

Authors:  Eileen K Jaffe; Linda Stith; Sarah H Lawrence; Mark Andrake; Roland L Dunbrack
Journal:  Arch Biochem Biophys       Date:  2013-01-11       Impact factor: 4.013

8.  Regulation of phenylalanine hydroxylase: conformational changes upon phosphorylation detected by H/D exchange and mass spectrometry.

Authors:  Jun Li; Paul F Fitzpatrick
Journal:  Arch Biochem Biophys       Date:  2013-03-26       Impact factor: 4.013

9.  The solution structure of the regulatory domain of tyrosine hydroxylase.

Authors:  Shengnan Zhang; Tao Huang; Udayar Ilangovan; Andrew P Hinck; Paul F Fitzpatrick
Journal:  J Mol Biol       Date:  2013-12-17       Impact factor: 5.469

10.  An additional substrate binding site in a bacterial phenylalanine hydroxylase.

Authors:  Judith A Ronau; Lake N Paul; Julian E Fuchs; Isaac R Corn; Kyle T Wagner; Klaus R Liedl; Mahdi M Abu-Omar; Chittaranjan Das
Journal:  Eur Biophys J       Date:  2013-07-17       Impact factor: 1.733
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