Modulation of the activity of newly synthesized human phenylalanine hydroxylase mutant proteins by low-molecular-weight compounds (Q46390774)

21 December 2017

title

Modulation of the activity of newly synthesized human phenylalanine hydroxylase mutant proteins by low-molecular-weight compounds (English)

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21 December 2017

Isabel Tavares de Almeida

author

3

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21 December 2017

João Leandro

object named as

João Leandro

2

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Paula Leandro

object named as

Paula Leandro

4

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author name string

Cátia Nascimento

1

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21 December 2017

publication date

1 September 2008

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21 December 2017

published in

The Protein Journal

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21 December 2017

volume

27

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21 December 2017

issue

6

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21 December 2017

page(s)

392-400

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21 December 2017

https://scigraph.springernature.com/pub.10.1007/s10930-008-9149-9

exact match

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cites work

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

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Chemical chaperones: a pharmacological strategy for disorders of protein folding and trafficking

21 January 2018

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Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease

21 January 2018

1 reference

Crossref

https://api.crossref.org/works/10.1007%2FS10930-008-9149-9

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

21 January 2018

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12 December 2020

Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency

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12 December 2020

Nucleotide sequence of a full-length complementary DNA clone and amino acid sequence of human phenylalanine hydroxylase

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The osmophobic effect: natural selection of a thermodynamic force in protein folding

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

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Chemical chaperones reduce endoplasmic reticulum stress and prevent mutant HFE aggregate formation

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12 December 2020

Clinical approach to treatable inborn metabolic diseases: an introduction

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

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Structure-function relationships of phenylalanine hydroxylase revealed by radiation target analysis.

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An overview of the role and diversity of compatible solutes in Bacteria and Archaea

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The V388M Mutation Results in a Kinetic Variant Form of Phenylalanine Hydroxylase

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Recombinant human phenylalanine hydroxylase: novel regulatory and structural properties.

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12 December 2020

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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Effects of polyhydroxy compounds on the structure and activity of alpha-chymotrypsin

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Chemical chaperone rescue of mutant human cystathionine beta-synthase

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Chemically chaperoning the actions of insulin

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Trimethylamine-N-oxide counteracts urea effects on rabbit muscle lactate dehydrogenase function: a test of the counteraction hypothesis

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The correlation of genotype and phenotype in Portuguese hyperphenylalaninemic patients.

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Osmolyte-induced folding enhances tryptic enzyme activity

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Trimethylamine-N-oxide-induced folding of alpha-synuclein.

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Phenylalanine 4-monooxygenase from rat liver

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The PAH gene, phenylketonuria, and a paradigm shift

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A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding

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12 December 2020

Glycerol increases the yield and activity of human phenylalanine hydroxylase mutant enzymes produced in a prokaryotic expression system.

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12 December 2020

Predicted effects of missense mutations on native-state stability account for phenotypic outcome in phenylketonuria, a paradigm of misfolding diseases

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12 December 2020

Effects of protein stabilizing agents on thermal backbone motions: a disulfide trapping study

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12 December 2020

Osmolytes induce structure in an early intermediate on the folding pathway of barstar

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12 December 2020

10.1007/S10930-008-9149-9

Identifiers

DOI

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21 December 2017

PubMed ID

18769885

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21 December 2017