Results 81 to 90 of about 109,987 (177)

Adult human liver mesenchymal progenitor cells express phenylalanine hydroxylase

, 2014
Phenylketonuria (PKU) is one of the most prevalent inherited metabolic diseases and is accountable for a severe encephalopathy by progressive intoxication of the brain by phenylalanine. This results from an ineffective L-phenylalanine hydroxylase enzyme (
Baruteau, Julien, Sokal, Etienne M, Nyabi, Omar, Najimi, Mustapha, Sokal, Etienne, Fauvart, Maarten   +5 more
core   +1 more source

Phenylketonuria and glycogen storage disease type III in sibs of one family

The Turkish Journal of Pediatrics, 2002
Hyperphenylalaninemia result from a block in the conversion of phenylalanine into tyrosine due to a defect in either the enzyme phenylalanine hydroxylase (98% of subjects) or in the metabolism of the cofactor tetrahydrobiopterin.
Tuncay Yilmazer, Albert H Van Gennip, Nico G G M Abeling, Imran Ozalp, Turgay Coşkun, Henk D Bakker   +5 more
doaj  

A unique dual activity amino acid hydroxylase in Toxoplasma gondii.

PLoS ONE, 2009
The genome of the protozoan parasite Toxoplasma gondii was found to contain two genes encoding tyrosine hydroxylase; that produces L-DOPA. The encoded enzymes metabolize phenylalanine as well as tyrosine with substrate preference for tyrosine.
Elizabeth A Gaskell, Judith E Smith, John W Pinney, Dave R Westhead, Glenn A McConkey   +4 more
doaj   +1 more source

Natural variation in a FtMYB32‐FtCES18 module drives Rhizoctonia solani resistance by reprogramming jasmonate and flavonoid metabolism in Tartary buckwheat

The Plant Journal, Volume 128, Issue 2, October 2026.
SUMMARY Tartary buckwheat (Fagopyrum tataricum) is renowned for its adaptability to complex growing conditions and stress tolerance. However, soil‐borne necrotrophic pathogens such as Rhizoctonia solani frequently cause devastating diseases that severely compromise its yield and quality. In this study, through a genome‐wide association study (GWAS), we
Yuanyuan Chen, Yang Liu, Xixu Peng, Haihua Wang, Zhengwu Fang, Jai Chand Rana, Nikhil Chrungoo, Maria D Logachev, Chaonan Guan, Meiliang Zhou   +9 more
wiley   +1 more source

Insights into the Catalytic Mechanisms of Phenylalanine and Tryptophan Hydroxylase from Kinetic Isotope Effects on Aromatic Hydroxylation^†

, 2016
Phenylalanine hydroxylase (PheH) and tryptophan hydroxylase (TrpH) catalyze the aromatic hydroxylation of phenylalanine and tryptophan, forming tyrosine and 5-hydroxytryptophan, respectively.
Jorge Alex Pavon (1994173), Paul F. Fitzpatrick (1340982)   +1 more
core   +1 more source

Relaxin‐2: Shaping the Proteomic Landscape of Skeletal Muscle Physiology, Glucose Trafficking, and Mitochondrial Function in Rat

The FASEB Journal, Volume 40, Issue 18, 30 September 2026.
Cardioprotective hormone relaxin‐2 showed relevant effects on rat skeletal muscle by altering proteins linked to muscle function, regeneration, differentiation, mitochondrial function, glucose metabolism, and structural integrity and organization. Specifically, relaxin‐2 reduced the expression of 95 proteins, increased 32, and elicited unique proteins ...
Xocas Vázquez‐Abuín, Lucía Gómez‐Cibeira, Javier García‐Seara, Pablo de la Fuente‐López, Carlos Minguito‐Carazo, Susana B. Bravo, Manuel Otero‐Santiago, Oreste Gualillo, José Ramón González‐Juanatey, Francisca Lago, Sandra Feijóo‐Bandín, Alana Aragón‐Herrera   +11 more
wiley   +1 more source

Essential role of the N-terminal autoregulatory sequence in the regulation of phenylalanine hydroxylase

, 2001
Phenylalanine hydroxylase (PAH) is activated by its substrate phenylalanine and inhibited by its cofactor tetrahydrobiopterin (BH4). The crystal structure of PAH revealed that the N-terminal sequence of the enzyme (residues 19-29) partially covered the ...
Jennings, I. G., Ian G. Jennings, Trazel Teh, Bostjan Kobe, Teh, T., Kobe, B.   +5 more
core   +1 more source

Thermal profiling reveals phenylalanine hydroxylase as an off-target of panobinostat [PDF]

, 2016
We describe a two-dimensional thermal proteome profiling strategy that can be combined with an orthogonal chemoproteomics approach to enable comprehensive target profiling of the marketed histone deacetylase inhibitor panobinostat. The N-hydroxycinnamide
Isabelle Becher, Marcus Bantscheff, Berkers, Celia R., Thilo Werner, Tögel, Ina, Zaal, Esther A., Bantscheff, Marcus, Savitski, Mikhail M., Fitzpatrick, Paul F., Mikhail M Savitski, Crystal A Khan, Salzer, Elsa, Muelbaier, Marcel, Ina Tögel, Paul F Fitzpatrick, Carola Doce, Rueger, Anne, Werner, Thilo, Doce, Carola, Sub Biomol.Mass Spectrometry & Proteom., Khan, Crystal A., Biomolecular Mass Spectrometry and Proteomics, Marcel Muelbaier, Becher, Isabelle, Elsa Salzer, Esther A Zaal, Celia R Berkers, Anne Rueger   +27 more
core   +1 more source

Case 67: Phenylalanine Hydroxylase: An Investigation of Its Structure Leads to the Development of PKU New Therapies [PDF]

, 2019
The characteristics of phenylalanine hydroxylase, the enzyme missing in persons afflicted with the genetic disorder phenylketonuria (PKU), are examined.
Cornely, Dr. Kathleen
core   +1 more source

Development of an Efficient Strategy to Improve Extracellular Polysaccharide Production of Ganoderma lucidum Using L-Phenylalanine as an Enhancer

Frontiers in Microbiology, 2019
Ganoderma lucidum has been a well-known species of basidiomycetes for a long time, and has been widely applied in the fields of food and medicine. Based on the simulation results of model iZBM1060 in our previous research, the effect of L-phenylalanine ...
Zhongbao Ma, Zhongbao Ma, Mengmeng Xu, Mengmeng Xu, Qiong Wang, Qiong Wang, Feng Wang, Huihua Zheng, Zhenghua Gu, Zhenghua Gu, Youran Li, Youran Li, Guiyang Shi, Guiyang Shi, Zhongyang Ding, Zhongyang Ding   +15 more
doaj   +1 more source