tetrahydrobiopterin


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tetrahydrobiopterin

[tet′rə·hī′drō·bī·op′tər·in]
a compound related to folic acid that functions as a coenzyme in the reactions hydroxylating phenylalanine, tryptophan, and tyrosine by carrying electrons to oxygen. Defects in its biosynthesis or regeneration affect all three hydroxylation reactions, interfere with production of the corresponding neurotransmitter precursors, and result in malignant hyperphenylalaninemia.

tetrahydrobiopterin

Metabolic disease A natural cofactor of aromatic amino acid hydroxylases and nitric oxide synthase, which may be used as an alternative to low-phenylalanine diets in Pts with mild phenylketonuria. See Phenylketonuria.

tetrahydrobiopterin

See PHENYLKETONURIA.

tetrahydrobiopterin

pterin cofactor in the mixed-function oxygenase, phenylalanine hydroxylase which catalyzes the conversion of phenylalanine to tyrosine. Hereditary deficiency of this enzyme is responsible for phenylketonuria (PKU).
References in periodicals archive ?
Effects of tetrahydrobiopterin on endothelial dysfunction in rats with ischemic acute renal failure.
Tetrahydrobiopterin (BH4) is an essential cofactor for tyrosine hydroxylase (TH), tryptophan hydroxylase, phenylalanine hydroxylase, and nitric-oxide synthase.
Tetrahydrobiopterin improves vascular endothelial function in ovariectomized rats.
GTP cyclohydrolase and tetrahydrobiopterin regulate pain sensitivity and persistence.
Background: Tetrahydrobiopterin (BH4) is an essential cofactor of nitric oxide synthases (NOSs) for the synthesis of nitric oxide (NO).
The cell lysate proteins were incubated in 20-mM Tris-HCl, containing FAD, tetrahydrobiopterin, and NADPH.
Tetrahydrobiopterin lowers muscle sympathetic nerve activity and improves augmentation index in patients with chronic kidney disease.
Critical role for tetrahydrobiopterin recycling by dihydrofolate reductase in regulation of endothelial nitric-oxide synthase coupling: Relative importance of the de novo biopterin synthesis versus salvage pathways.
Hwang, "Immobilization stress causes increases in tetrahydrobiopterin, dopamine, and neuromelanin and oxidative damage in the nigrostriatal system," Journal of Neurochemistry, vol.
4]: Tetrahydrobiopterin CIH: Chronic intermittent hypoxia DHE: Dihydroethidium ELISA: Enzyme-linked immunosorbent assay eNOS: Endothelial nitric oxide synthase [FIO.
In addition to Kuvan, the two companies agreed that Merck will return its option to develop and commercialize Peg-Pal, an investigational drug that is also designed for the treatment of PKU, an autosomal recessive genetic disorder caused by either a defect or a deficiency of the enzyme phenylalanine hydroxylase or its co-factor tetrahydrobiopterin.
12] also is involved in tetrahydrobiopterin synthesis in the brain, which is pivotal for synthesis of monoamine neurotransmitters.