Results 111 to 120 of about 14,507 (223)
Background: Sepiapterin is a natural precursor of tetrahydrobiopterin (BH4), a key cofactor for phenylalanine hydroxylase. It is being developed for the treatment of patients with phenylketonuria. In this study, the ethnic differences in pharmacokinetics
Lan Gao +4 more
doaj +1 more source
International audienceNitric oxide (NO) traffic within the reduced ferrous-nitrosyl complex of endothelial nitric-oxide synthase (eNOS) has been studied by ultrafast time-resolved absorption spectroscopy.
Berka, V. +7 more
core +1 more source
The Effects of Some Neurotoxins on Tetrahydrobiopterin Metabolism [PDF]
Previous studies in man have shown that following dosing with L--3,4-dihydroxyphenylalanine (L-DOPA) and cotrimoxazole, plasma biopterins were raised.
Edwards, Paul
core +1 more source
6-Pyruvoyl-tetrahydropterin synthase, which catalyzes the first step in the conversion of 7,8-dihydroneopterin triphosphate to tetrahydrobiopterin, was purified approximately 140,000-fold to apparent homogeneity from human liver.
Hans-Christoph CURTIUS +9 more
core +1 more source
Mini Review The metabolic and molecular bases of tetrahydrobiopterin-responsive phenylalanine hydroxylase deWciency [PDF]
About two-thirds of all mild phenylketonuria (PKU) patients are tetrahydrobiopterin (BH 4 )-responsive and thus can be potentially treated with BH 4 instead of a low-phenylalanine diet.
Nenad Blau, Heidi Erlandsen
core
L-dopa responsive dystonia due to sepiapterin reductase deficiency, a tetrahydrobiopterin deficiency presenting without hyperphenylalaninaemia [PDF]
Sepiapterin reductase (SPR) catalyses the last step in the biosynthesis of tetrahydrobiopterin (BH4), the essential cofactor for the aromatic amino acid hydroxylases as well as glycerol-ether monooxygenase and the three isoforms of nitric oxide ...
35th European Human Genetics Conference (ESHG) +5 more
core
Chapter 10: Tetrahydrobiopterin: An Essential Cofactor of Nitric Oxide Synthase with an Elusive Role
This chapter surveys the roles of pterins in biology and provides an understanding of biochemical pathways that determine tetrahydrobiopterin (BH4) levels in mammalian cells and how they are regulated.
Smith, C.L.
core
The biosynthesis of tetrahydrobiopterin from either dihydroneopterin triphosphate, sepiapterin, dihydrosepiapterin or dihydrobiopterin was investigated using extracts from human liver, dihydrofolate reductase and purified sepiapterin reductase from human
Heintel, Dorothee +5 more
core +1 more source
Tetrahydrobiopterin and dysfunction of endothelial nitric oxide synthase in coronary arteries.
BACKGROUND: The L-arginine/nitric oxide pathway plays a key role in the regulation of arterial tone. Biosynthesis of nitric oxide requires activation of nitric oxide synthase in the presence of tetrahydrobiopterin as a cofactor.
COSENTINO, Francesco, Katusić ZS
core

