Structural basis for ligand-dependent dimerization of phenylalanine hydroxylase regulatory domain

Structural basis for ligand-dependent dimerization of phenylalanine hydroxylase regulatory domain

The multi-domain enzyme phenylalanine hydroxylase (PAH) catalyzes the hydroxylation of dietary I-phenylalanine (Phe) to I-tyrosine. Inherited mutations that result in PAH enzyme deficiency are the genetic cause of the autosomal recessive disorder phenylketonuria. Phe is the substrate for the PAH act...

Volledige beschrijving

Bibliografische gegevens
Hoofdauteurs:	Patel, D, Kopec, J, Fitzpatrick, F, McCorvie, T, Yue, W
Formaat:	Journal article
Gepubliceerd in:	Nature Publishing Group 2016

Gelijkaardige items

Structural features of the regulatory ACT domain of phenylalanine hydroxylase.
door: Carla Carluccio, et al.
Gepubliceerd in: (2013-01-01)

Dynamic regulation of phenylalanine hydroxylase
door: Fuchs Julian E., et al.
Gepubliceerd in: (2014-07-01)

Structural basis of prolyl hydroxylase domain inhibition by Molidustat
door: Figg, WD, et al.
Gepubliceerd in: (2021)

Genotypes of patients with phenylalanine hydroxylase deficiency in the Wisconsin Amish
door: Jessica Scott Schwoerer, et al.
Gepubliceerd in: (2018-06-01)

High Protein Diet and Phenylalanine Hydroxylase Activities in Rats
door: Carty Michael P., et al.
Gepubliceerd in: (1989-11-01)

Phenylalanine hydroxylase mRNA rescues the phenylketonuria phenotype in mice
door: Maximiliano L. Cacicedo, et al.
Gepubliceerd in: (2022-10-01)

Mutations of the phenylalanine hydroxylase gene in patients with phenylketonuria in Shanxi, China
door: Yong-An Zhou, et al.
Gepubliceerd in: (2012-01-01)

Optical Coherence Tomography to Assess Neurodegeneration in Phenylalanine Hydroxylase Deficiency
door: Amelie S. Lotz-Havla, et al.
Gepubliceerd in: (2021-12-01)

Engineered Escherichia coli platforms for tyrosine-derivative production from phenylalanine using phenylalanine hydroxylase and tetrahydrobiopterin-regeneration system
door: Yasuharu Satoh, et al.
Gepubliceerd in: (2023-07-01)

Structural basis for oxygen degradation domain selectivity of the HIF prolyl hydroxylases
door: Chowdhury, R, et al.
Gepubliceerd in: (2016)

Structural basis for oxygen degradation domain selectivity of the HIF prolyl hydroxylases
door: Rasheduzzaman Chowdhury, et al.
Gepubliceerd in: (2016-08-01)

Screening and mutation analysis of phenylalanine hydroxylase deficiency in newborns from Jiangxi province
door: Baitao Zeng, et al.
Gepubliceerd in: (2023-02-01)

The analysis of the phenylalanine hydroxylase gene mutations by sequencing and ARMS techniques in Turkish patients
door: Umit Luleyap, et al.
Gepubliceerd in: (2016-12-01)

Deubiquitinase USP19 extends the residual enzymatic activity of phenylalanine hydroxylase variants
door: Neha Sarodaya, et al.
Gepubliceerd in: (2022-08-01)

Phenylalanine 4-Hydroxylase Contributes to Endophytic Bacterium Pseudomonas fluorescens’ Melatonin Biosynthesis
door: Jian Jiao, et al.
Gepubliceerd in: (2021-11-01)

Evidence of the Phenylalanine Hydroxylase Involvement In Biosynthesis of Pteridines and Hydroxylation of Tryptophan in Drosophila melanogaster
door: Alcaniz S., et al.
Gepubliceerd in: (1995-08-01)

Chronic Immune Stimulation May Cause Moderate Impairment of Phenylalanine 4-hydroxylase
door: Scholl-Bürgi S, et al.
Gepubliceerd in: (2011-02-01)

The effect of tetrahydrobiopterin on the thermostability of human phenylalanine hydroxylase as studied by circular dichroism spectroscopy
door: Thórólfsson Matthias, et al.
Gepubliceerd in: (2000-05-01)

The phenylalanine hydroxylase locus: A marker for the history of phenylketonuria and human genetic diversity - Discussion
door: Beighton, P, et al.
Gepubliceerd in: (1996)

Modulation of Human Phenylalanine Hydroxylase by 3-Hydroxyquinolin-2(1H)-One Derivatives
door: Raquel R. Lopes, et al.
Gepubliceerd in: (2021-03-01)

The spectrum of phenylalanine hydroxylase variants and genotype–phenotype correlation in phenylketonuria patients in Gansu, China
door: Chuan Zhang, et al.
Gepubliceerd in: (2023-04-01)

Strategies for Successful Long-Term Engagement of Adults With Phenylalanine Hydroxylase Deficiency Returning to the Clinic
door: Janet Thomas MD, et al.
Gepubliceerd in: (2017-09-01)

Structural Properties of Phenylalanine-Based Dimers Revealed Using IR Action Spectroscopy
door: Iuliia Stroganova, et al.
Gepubliceerd in: (2022-04-01)

Solation of phenylalanine ammonia lyase and cinnamate 4-hydroxylase genes from bayur medicinal plant /
door: Aimi Amirah Abd Ajis, 1992-, author
Gepubliceerd in: (2018)

Solation of phenylalanine ammonia lyase and cinnamate 4-hydroxylase genes from bayur medicinal plant /
door: Aimi Amirah Abd Ajis, 1992-, author, et al.
Gepubliceerd in: (2018)

P029: The effects of the pharmacological chaperone sepiapterin on phenylalanine hydroxylase activity of common variants in phenylketonuria*
door: Søren Gersting, et al.
Gepubliceerd in: (2023-01-01)

Impact of Fluorinated Ionic Liquids on Human Phenylalanine Hydroxylase—A Potential Drug Delivery System
door: Márcia M. S. Alves, et al.
Gepubliceerd in: (2022-03-01)

Molecular assessment and bioinformatic analysis of two common mutations of phenylalanine hydroxylase (PAH) gene by HRM
door: M Amir, et al.
Gepubliceerd in: (2017-06-01)

Gene mutations in children with phenylalanine hydroxylase deficiency: an analysis of 45 cases in some regions of Chongqing
door: WANG Dongjuan, et al.
Gepubliceerd in: (2021-05-01)

Dynamic inter-domain transformations mediate the allosteric regulation of human 5, 10-methylenetetrahydrofolate reductase
door: Linnea K. M. Blomgren, et al.
Gepubliceerd in: (2024-04-01)

Substituting Tyr138 in the active site loop of human phenylalanine hydroxylase affects catalysis and substrate activation
door: João Leandro, et al.
Gepubliceerd in: (2017-07-01)

Genetic Diversity and Balancing Selection within the Human Phenylalanine Hydroxylase (PAH) Gene Region in Iranian Population
door: A Haghighatnia, et al.
Gepubliceerd in: (2012-05-01)

Genetic Diversity and Balancing Selection within the Human Phenylalanine Hydroxylase (PAH) Gene Region in Iranian Population
door: J Mowla, et al.
Gepubliceerd in: (2012-04-01)

Quantitative analysis by flow cytometry of green fluorescent protein-tagged human phenylalanine hydroxylase expressed in Dictyostelium
door: Kim Hye-Lim, et al.
Gepubliceerd in: (2017-12-01)

Phenylalanine Hydroxylase RNAi Knockdown Negatively Affects Larval Development, Molting and Swimming Performance of Salmon Lice
door: Prashanna Guragain, et al.
Gepubliceerd in: (2020-12-01)

Assessing the accuracy of California county level COVID-19 hospitalization forecasts to inform public policy decision making
door: Lauren A. White, et al.
Gepubliceerd in: (2023-04-01)

Structural basis for binding of the renal carcinoma target hypoxia-inducible factor 2α to prolyl hydroxylase domain 2
door: Figg, WD, et al.
Gepubliceerd in: (2023)

Engineered phenylalanine hydroxylase coupled with an effective cofactor synthesis and regeneration system for high-yield production of 5-hydroxytryptophan
door: Yulin Ai, et al.
Gepubliceerd in: (2025-03-01)

Investigation of exon 4 mutations of phenylalanine hydroxylase gene in phenylketonuria patients in Guilan Province using PCR-sequencing
door: Neda Pourvatan, et al.
Gepubliceerd in: (2019-02-01)

The Predictive Value of Genetic Analyses in the Diagnosis of Tetrahydrobiopterin (BH4)-Responsiveness in Chinese Phenylalanine Hydroxylase Deficiency Patients
door: Tianwen Zhu, et al.
Gepubliceerd in: (2017-07-01)