Cargando…

Modulation of Human Phenylalanine Hydroxylase by 3-Hydroxyquinolin-2(1H)-One Derivatives

Phenylketonuria (PKU) is a genetic disease caused by deficient activity of human phenylalanine hydroxylase (hPAH) that, when untreated, can lead to severe psychomotor impairment. Protein misfolding is recognized as the main underlying pathogenic mechanism of PKU. Therefore, the use of stabilizers of...

Descripción completa

Detalles Bibliográficos
Autores principales:	Lopes, Raquel R., Tomé, Catarina S., Russo, Roberto, Paterna, Roberta, Leandro, João, Candeias, Nuno R., Gonçalves, Lídia M. D., Teixeira, Miguel, Sousa, Pedro M. F., Guedes, Rita C., Vicente, João B., Gois, Pedro M. P., Leandro, Paula
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2021
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8003416/ https://www.ncbi.nlm.nih.gov/pubmed/33808760 http://dx.doi.org/10.3390/biom11030462

Ejemplares similares

Structure of full-length wild-type human phenylalanine hydroxylase by small angle X-ray scattering reveals substrate-induced conformational stability
por: Tomé, Catarina S., et al.
Publicado: (2019)

Author Correction: Structure of full-length wild-type human phenylalanine hydroxylase by small angle X-ray scattering reveals substrate-induced conformational stability
por: Tomé, Catarina S., et al.
Publicado: (2019)

PKU mutation p.G46S prevents the stereospecific binding of l‐phenylalanine to the dimer of human phenylalanine hydroxylase regulatory domain
por: Leandro, João, et al.
Publicado: (2017)

Substituting Tyr(138) in the active site loop of human phenylalanine hydroxylase affects catalysis and substrate activation
por: Leandro, João, et al.
Publicado: (2017)

Phenylalanine Binding Is Linked to Dimerization of the Regulatory Domain of Phenylalanine Hydroxylase
por: Zhang, Shengnan, et al.
Publicado: (2014)

Activation of Phenylalanine Hydroxylase by Phenylalanine Does Not Require Binding in the Active Site
por: Roberts, Kenneth M., et al.
Publicado: (2014)

Impact of Fluorinated Ionic Liquids on Human Phenylalanine Hydroxylase—A Potential Drug Delivery System
por: Alves, Márcia M. S., et al.
Publicado: (2022)

Phenylalanine Hydroxylase Misfolding and Pharmacological Chaperones
por: Underhaug, Jarl, et al.
Publicado: (2012)

Dynamic Regulation of Phenylalanine Hydroxylase by Simulated Redox Manipulation
por: Fuchs, Julian E., et al.
Publicado: (2012)

Recommendations for the nutrition management of phenylalanine hydroxylase deficiency
por: Singh, Rani H., et al.
Publicado: (2014)

Alchemical Design of Pharmacological Chaperones with Higher Affinity for Phenylalanine Hydroxylase
por: Conde-Giménez, María, et al.
Publicado: (2022)

Structural Features of the Regulatory ACT Domain of Phenylalanine Hydroxylase
por: Carluccio, Carla, et al.
Publicado: (2013)

Mutations of the phenylalanine hydroxylase gene in Iranian patients with phenylketonuria
por: Biglari, Alireza, et al.
Publicado: (2015)

Genotypes of patients with phenylalanine hydroxylase deficiency in the Wisconsin Amish
por: Schwoerer, Jessica Scott, et al.
Publicado: (2018)

Stabilization of tryptophan hydroxylase 2 by l‐phenylalanine‐induced dimerization
por: Tidemand, Kasper D., et al.
Publicado: (2016)

Characterization of phenylalanine hydroxylase gene variants and analysis of genotype–phenotype correlation in patients with phenylalanine hydroxylase deficiency from Fujian Province, Southeastern China
por: Zhou, Jinfu, et al.
Publicado: (2022)

Optical Coherence Tomography to Assess Neurodegeneration in Phenylalanine Hydroxylase Deficiency
por: Lotz-Havla, Amelie S., et al.
Publicado: (2021)

A Conserved Acidic Residue in Phenylalanine Hydroxylase Contributes to Cofactor Affinity and Catalysis
por: Ronau, Judith A., et al.
Publicado: (2014)

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

Phenylalanine Hydroxylase Deficiency and Citrin Deficiency in a Chinese Infant
por: Ye, Jun, et al.
Publicado: (2015)

Mutation analysis of Phenylalanine hydroxylase gene in Iranian patients with Phenylketonuria
por: Rastegar Moghadam, Mahsa, et al.
Publicado: (2018)

Structural and Functional Impact of Seven Missense Variants of Phenylalanine Hydroxylase
por: Pecimonova, Martina, et al.
Publicado: (2019)

Phenylalanine hydroxylase mRNA rescues the phenylketonuria phenotype in mice
por: Cacicedo, Maximiliano L., et al.
Publicado: (2022)

Glycerol as an Efficient Medium for the Petasis Borono–Mannich Reaction
por: Rosholm, Tomi, et al.
Publicado: (2015)

Diazaborines as New Inhibitors of Human Neutrophil Elastase
por: António, João P. M., et al.
Publicado: (2018)

Metal Coordination and Biological Screening of a Schiff Base Derived from 8-Hydroxyquinoline and Benzothiazole
por: Ribeiro, Nádia, et al.
Publicado: (2022)

Structural basis for ligand-dependent dimerization of phenylalanine hydroxylase regulatory domain
por: Patel, Dipali, et al.
Publicado: (2016)

Mutational and phenotypic spectrum of phenylalanine hydroxylase deficiency in Zhejiang Province, China
por: Chen, Ting, et al.
Publicado: (2018)

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

Structural and thermodynamic insight into phenylalanine hydroxylase from the human pathogen Legionella pneumophila()
por: Leiros, Hanna-Kirsti S., et al.
Publicado: (2013)

Phenylalanine 4-Hydroxylase Contributes to Endophytic Bacterium Pseudomonas fluorescens’ Melatonin Biosynthesis
por: Jiao, Jian, et al.
Publicado: (2021)

Unravelling the Complex Denaturant and Thermal-Induced Unfolding Equilibria of Human Phenylalanine Hydroxylase
por: Conde-Giménez, María, et al.
Publicado: (2021)

Deubiquitinase USP19 extends the residual enzymatic activity of phenylalanine hydroxylase variants
por: Sarodaya, Neha, et al.
Publicado: (2022)

Screening and mutation analysis of phenylalanine hydroxylase deficiency in newborns from Jiangxi province
por: Zeng, Baitao, et al.
Publicado: (2023)

Immunocytochemical identification of phenylalanine hydroxylase and albumin in cultured hepatoma cells and isolated rat hepatocytes
Publicado: (1981)

Birth prevalence of phenylalanine hydroxylase deficiency: a systematic literature review and meta-analysis
por: Foreman, Pamela K., et al.
Publicado: (2021)

The spectrum of phenylalanine hydroxylase variants and genotype–phenotype correlation in phenylketonuria patients in Gansu, China
por: Zhang, Chuan, et al.
Publicado: (2023)

Phenylalanine meta‐Hydroxylase: A Single Residue Mediates Mechanistic Control of Aromatic Amino Acid Hydroxylation
por: Grüschow, Sabine, et al.
Publicado: (2019)

Formation and Entrapment of Tris(8-hydroxyquinoline)aluminum from 8-Hydroxyquinoline in Anodic Porous Alumina
por: Yamaguchi, Shohei, et al.
Publicado: (2016)

The Amino Acid Specificity for Activation of Phenylalanine Hydroxylase Matches the Specificity for Stabilization of Regulatory Domain Dimers
por: Zhang, Shengnan, et al.
Publicado: (2015)

Cannot write session to /tmp/vufind_sessions/sess_32dh80vh88jhoah3vja37u2gtd