Phenylalanine

phenylalanine hydroxylase ; Homo sapiens







246 Article(s)
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1 34952194 Phenylalanine hydroxylase (PAH) plays a positive role during WSSV and Vibrio parahaemolyticus infection in Litopenaeus vannamei. 2022 Jan 1
2 35325557 Evaluation of the Consumption of Fruits and Vegetables by Phenylketonurics in the Metabolic Control of Phenylalanine: An Integrative Review. 2022 May 6
3 35356682 Development of an mRNA replacement therapy for phenylketonuria. 2022 Jun 14 1
4 35449354 Deubiquitinase USP19 enhances phenylalanine hydroxylase protein stability and its enzymatic activity. 2022 Apr 21 2
5 35600090 Lipid nanoparticle delivers phenylalanine ammonia lyase mRNA to the liver leading to catabolism and clearance of phenylalanine in a phenylketonuria mouse model. 2022 Sep 1
6 33221376 Manipulation of a cation-π sandwich reveals conformational flexibility in phenylalanine hydroxylase. 2021 Apr 1
7 33465300 Phenylketonuria in Portugal: Genotype-phenotype correlations using molecular, biochemical, and haplotypic analyses. 2021 Mar 1
8 33485250 Adsorption of organic molecules on carbon surfaces: Experimental data and molecular dynamics simulation considering multiple protonation states. 2021 May 1
9 33819046 Plasma Phospholipidomic Profile Differs between Children with Phenylketonuria and Healthy Children. 2021 May 7 2
10 34057292 Pharmacokinetic, pharmacodynamic, and immunogenic rationale for optimal dosing of pegvaliase, a PEGylated bacterial enzyme, in adult patients with phenylketonuria. 2021 Sep 1
11 34145024 A phosphoproteomic approach reveals that PKD3 controls PKA-mediated glucose and tyrosine metabolism. 2021 Aug 1
12 34162223 Dysregulated Phenylalanine Catabolism Plays a Key Role in the Trajectory of Cardiac Aging. 2021 Aug 17 2
13 34207146 Unravelling the Complex Denaturant and Thermal-Induced Unfolding Equilibria of Human Phenylalanine Hydroxylase. 2021 Jun 18 2
14 34412683 A retrospective study on disease management in children and adolescents with phenylketonuria during the Covid-19 pandemic lockdown in Austria. 2021 Aug 19 4
15 34784942 Italian national consensus statement on management and pharmacological treatment of phenylketonuria. 2021 Nov 16 3
16 34819582 Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria. 2021 Nov 24 2
17 34900593 Untreated PKU patients without intellectual disability: SHANK gene family as a candidate modifier. 2021 Dec 1
18 35082602 Engineering Organoids for in vitro Modeling of Phenylketonuria. 2021 1
19 31883647 5-year retrospective analysis of patients with phenylketonuria (PKU) and hyperphenylalaninemia treated at two specialized clinics. 2020 Mar 4
20 32106880 Lower plasma cholesterol, LDL-cholesterol and LDL-lipoprotein subclasses in adult phenylketonuria (PKU) patients compared to healthy controls: results of NMR metabolomics investigation. 2020 Feb 27 3
21 32217972 Management of Women With Phenylalanine Hydroxylase Deficiency (Phenylketonuria): ACOG Committee Opinion Summary, Number 802. 2020 Apr 1
22 32450880 Saccadic reaction time and ocular findings in phenylketonuria. 2020 May 25 1
23 32668217 The Genetic Landscape and Epidemiology of Phenylketonuria. 2020 Aug 6 1
24 32742934 Unmet needs in PKU and the disease impact on the day-to-day lives in Brazil: Results from a survey with 228 patients and their caregivers. 2020 Sep 3
25 32883979 Dietary intake and nutritional status of patients with phenylketonuria in Taiwan. 2020 Sep 3 1
26 33101986 Phenylketonuria in the Latvian population: Molecular basis, phenylalanine levels, and patient compliance. 2020 Dec 3
27 33260674 E3 Ubiquitin Ligase APC/CCdh1 Regulation of Phenylalanine Hydroxylase Stability and Function. 2020 Nov 28 1
28 33335942 Long-Term Metabolic Correction of Phenylketonuria by AAV-Delivered Phenylalanine Amino Lyase. 2020 Dec 11 1
29 30504004 Transient phenylketonuria in premature infants. 2019 Mar 2
30 30648773 Toward mechanistic models for genotype-phenotype correlations in phenylketonuria using protein stability calculations. 2019 Apr 1
31 30674554 The phenylketonuria-associated substitution R68S converts phenylalanine hydroxylase to a constitutively active enzyme but reduces its stability. 2019 Mar 22 1
32 30864096 Phenylketonuria: Current Treatments and Future Developments. 2019 Apr 1
33 30992364 Tau repeat regions contain conserved histidine residues that modulate microtubule-binding in response to changes in pH. 2019 May 31 1
34 31038957 Uncovered Dynamic Coupling Resolves the Ambiguous Mechanism of Phenylalanine Hydroxylase Oxygen Binding. 2019 May 30 4
35 31076506 Biophysical characterization of full-length human phenylalanine hydroxylase provides a deeper understanding of its quaternary structure equilibrium. 2019 Jun 28 2
36 31105574 Propagation of Plasma L-Phenylalanine Concentration Fluctuations to the Neurovascular Unit in Phenylketonuria: An in silico Study. 2019 1
37 31118288 Structure of full-length human phenylalanine hydroxylase in complex with tetrahydrobiopterin. 2019 Jun 4 2
38 31434173 Catalytic Ability Improvement of Phenylalanine Hydroxylase from Chromobacterium violaceum by N-Terminal Truncation and Proline Introduction. 2019 Sep 28 1
39 29454221 13C-phenylalanine breath test and serum biopterin in schizophrenia, bipolar disorder and major depressive disorder. 2018 Apr 2
40 29653686 Pegvaliase for the treatment of phenylketonuria: Results of a long-term phase 3 clinical trial program (PRISM). 2018 May 1
41 29777816 Aptamer-based assay for monitoring genetic disorder phenylketonuria (PKU). 2018 Sep 1
42 29909188 Late-diagnosed phenylketonuria mimicking x-linked adrenoleukodystrophy with heterozygous mutations of the PAH Gene: A case report and literature review. 2018 Aug 3
43 30201326 A bone mineralization defect in the Pahenu2 model of classical phenylketonuria involves compromised mesenchymal stem cell differentiation. 2018 Nov 1
44 30287685 Simulations of the regulatory ACT domain of human phenylalanine hydroxylase (PAH) unveil its mechanism of phenylalanine binding. 2018 Dec 21 10
45 30346142 Phosphorylation of Phenylalanine Hydroxylase Increases the Rate Constant for Formation of the Activated Conformation of the Enzyme. 2018 Nov 6 2
46 30578407 Conformational selection turns on phenylalanine hydroxylase. 2018 Dec 21 1
47 28174686 PKU mutation p.G46S prevents the stereospecific binding of l-phenylalanine to the dimer of human phenylalanine hydroxylase regulatory domain. 2017 Feb 5
48 28182360 Mutation Analysis in Classical Phenylketonuria Patients Followed by Detecting Haplotypes Linked to Some PAH Mutations. 2017 Feb 1 1
49 28389235 DNA methylated alleles of the phenylalanine hydroxylase promoter remodeled at elevated phenylalanine levels in newborns with hyperphenylalaninemia. 2017 Aug 2
50 28593914 Improved metabolic control in tetrahydrobiopterin (BH4), responsive phenylketonuria with sapropterin administered in two divided doses vs. a single daily dose. 2017 Jul 26 1