Results 51 to 60 of about 32,654 (265)

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   +4 more sources

In Vivo Biosynthesis and Direct Incorporation of Non‐Canonical Amino Acids into Proteins

ChemBioChem, Accepted Article.
Autonomous cells are engineered biological systems capable of biosynthesising and directly incorporating non‐canonical amino acids (ncAAs) into proteins. These systems have the potential to extend the applicability of the genetic code to enable large‐scale fermentative production of proteins carrying ncAAs.
Ivana Drienovska, Jan Hendrik Illies, T. Moritz Weber   +2 more
wiley   +1 more source

Ethanol triggers grape gene expression leading to anthocyanin accumulation during berry ripening [PDF]

, 2002
Recent studies have shown that low doses of ethanol stimulate the maturation of some fruits. The present work showed that spraying Cabernet Sauvignon grapes, with 5% ethanol at veraison enhances the anthocyanin accumulation.
Chervin, Christian, El-Kereamy, Ashraf, Ford, Christopher, Mondies, Hélène, Monje, Marie-Carmen, Moutounet, Michel, Nepveu, Françoise, Roustan, Jean-Paul, Souquet, Jean-Marc, Van Heeswijck, R.   +9 more
core   +2 more sources

Allosteric regulation of phenylalanine hydroxylase [PDF]

Archives of Biochemistry and Biophysics, 2012
The liver enzyme phenylalanine hydroxylase is responsible for conversion of excess phenylalanine in the diet to tyrosine. Phenylalanine hydroxylase is activated by phenylalanine; this activation is inhibited by the physiological reducing substrate tetrahydrobiopterin.
openaire   +3 more sources

Seizures and electroencephalographic findings in inborn errors of metabolism: Clues to differential diagnosis in the neonatal period, infancy, childhood and adolescence, and review of the literature

Epileptic Disorders, EarlyView.
Abstract Although inborn errors of metabolism (IEM) are a rare cause of epilepsy, seizures are a common presentation in these disorders. Seizures in IEM are frequently refractory to conventional anti‐seizure medication and might warrant initiation of specific treatments based on vitamins or dietary modifications or provision of alternative substrates ...
D. Kapoor, S. Sharma, A. Kaminska, R. Nabbout, N. Chemaly, M. Schiff, P. De Lonlay, M. Eisermann   +7 more
wiley   +1 more source

Hyperphenylalaninemia and serotonin deficiency in Dnajc12-deficient mice

Communications Biology
Serotonin exerts numerous neurological and physiological actions in the brain and in the periphery. It is generated by two different tryptophan hydroxylase enzymes, TPH1 and TPH2, in the periphery and in the brain, respectively, which are members of the ...
Yunqing Cao, Oliver Popp, Niccolo Milani, Fatimunnisa Qadri, Ralf Kühn, Philipp Mertins, Michael Bader, Natalia Alenina   +7 more
doaj   +1 more source

ZZE-Configuration of chromophore ß-153 in C-phycocyanin from Mastigocladus laminosus [PDF]

, 1987
The photochemistry of C-phycocyanin has been studied after denaturation in the dark. It shows an irreversible reaction which has characteristics of a Ζ,Ζ,Ε- to Z,Z,Z-isomerization of dihydrobilins. Its amplitude depends on the reaction conditions, with
Bode, W., Fischer, R., Huber, R., Rüdiger, W., Scheer, Hugo, Schirmer, T., Schmidt, G., Siebzehnrübl, S.   +7 more
core   +1 more source

Inherited metabolic epilepsies–established diseases, new approaches

Epilepsia Open, EarlyView.
Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
wiley   +1 more source

High Protein Diet and Phenylalanine Hydroxylase Activities in Rats

Pteridines, 1989
The effects of a diet of 85% casein on the activities of the phenylalanine hydroxylases of rat liver and kidney have been compared. Whereas only the tetrahydrobiopterin-dependent activity of rat hepatic phenylalanine hydroxylase is significantly ...
Carty Michael P., Beirne Edel, Donlon John   +2 more
doaj   +1 more source

A method for phenylalanine self-monitoring using phenylalanine ammonia-lyase and a pre-existing portable ammonia detection system

Molecular Genetics and Metabolism Reports, 2023
Phenylketonuria is an inborn error of phenylalanine metabolism caused by a phenylalanine hydroxylase deficiency. To prevent the occurrence of neurological symptoms and maternal complications resulting from phenylketonuria, patients must adhere to a ...
Yoichi Wada, Eriko Totsune, Yasuko Mikami-Saito, Atsuo Kikuchi, Toshio Miyata, Shigeo Kure   +5 more
doaj   +1 more source