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 ?
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.
PKU is an autosomal recessive genetic disorder caused by a defect or a deficiency of the enzyme phenylalanine hydroxylase (PAH) or its cofactor tetrahydrobiopterin (BH4).
5-methyltetrahydrofolate rapidly improves endothelial function and decreases superoxide production in human vessels: effects on vascular tetrahydrobiopterin availability and endothelial nitric oxide synthase coupling.
GCH1 is needed to synthesize the protein tetrahydrobiopterin (BH4), and people with GCH1 variants produce less BH4 after nerve injury.
To support this notion, a few studies have demonstrated that the expressions of NOS and tetrahydrobiopterin (a NOS cofactor) are regulated by CREB protein-dependent mechanisms (Boissel et al.
In a study that randomized 46 children to tetrahydrobiopterin or placebo for 16 weeks (J.
The GCH1 enzyme is involved in the rate-limiting step of tetrahydrobiopterin (BH4) synthesis (Fig.
Decreased arteriolar tetrahydrobiopterin is linked to superoxide generation from nitric oxide synthase in mice fed high salt.
Dudley and his colleagues knew that-in blood vessels-the problem was depletion of a chemical called tetrahydrobiopterin, or BH4, which is needed for the tissues to make NO.
Folates are alleged to participate in the endogenous regeneration of tetrahydrobiopterin (Wever et al.
Therapeutic use: Sapropterin (24) is a biologically active synthetic form of tetrahydrobiopterin (BH4) indicated to reduce blood phenylalanine levels in patients with hyperphenylalaninemia due to BH4-responsive phenylketonuria.