Results 21 to 30 of about 24,432 (224)

Dynamic regulation of phenylalanine hydroxylase

Pteridines, 2014
Phenylalanine hydroxylase (PAH) is the key enzyme in phenylalanine metabolism, catalyzing its oxidative breakdown to tyrosine. Its function in the committed step of amino acid metabolism requires strict regulation. Thus, several regulatory mechanisms are
Fuchs Julian E., Fuchs Dietmar, Liedl Klaus R.   +2 more
doaj   +1 more source

Biopterin responsive phenylalanine hydroxylase deficiency [PDF]

Genetics in Medicine, 2004
Phenylketonuria (PKU) is an autosomal recessive disorder caused by mutations in the phenylalanine hydroxylase (PAH) gene. There have been more than 400 mutations identified in the PAH gene leading to variable degrees of deficiency in PAH activity, and consequently a wide spectrum of clinical severity.
Reuben, Matalon, Richard, Koch, Kimberlee, Michals-Matalon, Kathryn, Moseley, Sankar, Surendran, Stephen, Tyring, Heidi, Erlandsen, Alejandra, Gamez, Raymond C, Stevens, Anne, Romstad, Lisbeth B, Møller, Flemming, Guttler   +11 more
openaire   +2 more sources

Dynamic regulation of phenylalanine hydroxylase by simulated redox manipulation.

PLoS ONE, 2012
Recent clinical studies revealed increased phenylalanine levels and phenylalanine to tyrosine ratios in patients suffering from infection, inflammation and general immune activity.
Julian E Fuchs, Roland G Huber, Susanne von Grafenstein, Hannes G Wallnoefer, Gudrun M Spitzer, Dietmar Fuchs, Klaus R Liedl   +6 more
doaj   +1 more source

Liver phenylalanine hydroxylase assay [PDF]

Biochemical Medicine, 1976
The first reaction is catalyzed by phenylalanine hydroxylase and the second reaction, which generates the reduced form of pteridine cofactor (biopterin), is catalyzed by dihydropteridine reductase (l-3). A direct assay of phenylalanine hydroxylase can be achieved by supplying optimal concentrations of reduced pteridine cofactor or an analog of the ...
openaire   +3 more sources

Use of sapropterin in Mexican patients with yperphenylalaninemia

Acta Pediátrica de México, 2014
Hyperphenylalaninemia is caused by deficient enzyme activity of phenylalanine hydroxylase. It was one of the first genetic disorders susceptible to treatment with a natural protein restricted diet for life.
Susana Monroy-Santoyo, Leticia Belmont-Martínez, Cynthia Fernández-Lainez   +2 more
doaj   +1 more source

Phenylalanine 4-Hydroxylase Contributes to Endophytic Bacterium Pseudomonas fluorescens’ Melatonin Biosynthesis

Frontiers in Genetics, 2021
Melatonin acts both as an antioxidant and as a growth regulatory substance in plants. Pseudomonas fluorescens endophytic bacterium has been shown to produce melatonin and increase plant resistance to abiotic stressors through increasing endogenous ...
Jian Jiao, Jian Jiao, Yan Xia, Yingli Zhang, Xueli Wu, Chonghuai Liu, Jiancan Feng, Xianbo Zheng, Shangwei Song, Tuanhui Bai, Chunhui Song, Miaomiao Wang, Hongguang Pang   +12 more
doaj   +1 more source

Incidence of Phenylketonuria in Southern Iran [PDF]

Iranian Journal of Medical Sciences, 2010
Background: Phenylketonuria is a hereditary, autosomal recessivedisorder caused by deficiency of phenylalanine hydroxylaseor its cofactor tetrahydrobiopterin.
Asadollah Habib, Mohammad Hossein Fallahzadeh, Hamid Reza Kazeroni, Amir Hossein Ganjkarimi   +3 more
doaj  

Investigation of five common mutations on Phenylalanine Hydroxylase Gene of Phenylketonuria Patients from Two Provinces in North of Iran

International Journal of Preventive Medicine, 2017
Background: There are more than 500 different mutations on phenylalanine hydroxylase (PAH) gene that is responsible for phenylketonuria (PKU) diseases and the spectrum of these mutations is varied in different populations. The main clinical manifestation
Daniel Zamanfar, Hossein Jalali, Mohammad Reza Mahdavi, Morteza Maadanisani, Hossein Zaeri, Eynollah Asadpoor   +5 more
doaj   +1 more source

Engineered Escherichia coli platforms for tyrosine-derivative production from phenylalanine using phenylalanine hydroxylase and tetrahydrobiopterin-regeneration system

Biotechnology for Biofuels and Bioproducts, 2023
Background Aromatic compounds derived from tyrosine are important and diverse chemicals that have industrial and commercial applications. Although these aromatic compounds can be obtained by extraction from natural producers, their growth is slow, and ...
Yasuharu Satoh, Keita Fukui, Daisuke Koma, Ning Shen, Taek Soon Lee   +4 more
doaj   +1 more source

Disturbed amino acid metabolism in HIV: association with neuropsychiatric symptoms

Frontiers in Psychiatry, 2015
Blood levels of the amino acid phenylalanine, as well as of the tryptophan breakdown product kynurenine, are found to be elevated in human immunodeficiency virus type 1 (HIV-1)-infected patients.
Johanna M Gostner, Kathrin eBecker, Katharina eKurz, Dietmar eFuchs   +3 more
doaj   +1 more source