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...

Disgrifiad llawn

Manylion Llyfryddiaeth
Prif Awduron:	Patel, D, Kopec, J, Fitzpatrick, F, McCorvie, T, Yue, W
Fformat:	Journal article
Cyhoeddwyd:	Nature Publishing Group 2016

Eitemau Tebyg

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

Dynamic regulation of phenylalanine hydroxylase
gan: Fuchs Julian E., et al.
Cyhoeddwyd: (2014-07-01)

Structural basis of prolyl hydroxylase domain inhibition by Molidustat
gan: Figg, WD, et al.
Cyhoeddwyd: (2021)

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

The oxygen sensor prolyl hydroxylase domain 2 regulates the in vivo suppressive capacity of regulatory T cells
gan: Yousra Ajouaou, et al.
Cyhoeddwyd: (2022-02-01)

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

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