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Sapropterin dihydrochloride, 6-R-L-erythro-5,6,7,8-tetrahydrobiopterin, in the treatment of phenylketonuria

Expert Opinion on Investigational Drugs, 2008
Sapropterin dihydrochloride, 6-R-L-erythro-5,6,7,8-tetrahydrobiopterin (BH4) is being introduced in the US for treatment of phenylketonuria (PKU). This compound has been in use in Europe to treat mild forms of PKU. Tetrahydrobiopterin is the cofactor in the hydroxylation reaction of the three aromatic amino acids phenylalanine, tyrosine and tryptophan.
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6R-L-erythro-5,6,7,8-tetrahydrobiopterin is involved in brain vulnerability of senescence-accelerated mouse during the processes of aging.

Growth, development, and aging : GDA, 1998
The effects of age on the dopamine (DA) turnover and 6R-L-erythro-5,6,7,8-tetrahydrobiopterin (6R-BH4), a cofactor for monooxygenases, were investigated in the striatum and midbrain of the senescence accelerated mouse (SAM) at 6, 12 and 15 months old. 6R-BH4 and DA levels were decreased significantly in the striatum of 15-month-old SAM-P/1 (senescence ...
K, Yoshimoto   +3 more
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6R-l-erythro-5,6,7,8-tetrahydrobiopterin (BH4): A potential treatment for all symptom domains of schizophrenia

Medical Hypotheses, 2014
Current psychopharmacological treatment of schizophrenia is suboptimal and the available antipsychotic medications have little or no effect on negative and cognitive symptom domains of the disorder. 6R-l-erythro-5,6,7,8-tetrahydrobiopterin (BH4) is a cofactor involved in the synthesis of dopamine, serotonin and nitric oxide which have all been ...
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Inhibition of Neuronal Nitric Oxide Synthase by 4-Amino Pteridine Derivatives:  Structure−Activity Relationship of Antagonists of (6R)-5,6,7,8-Tetrahydrobiopterin Cofactor

Journal of Medicinal Chemistry, 1999
The family of nitric oxide synthases (NOS) catalyzes the conversion of L-arginine to L-citrulline and nitric oxide (NO), an important cellular messenger molecule which has been implicated in the pathophysiology of septic shock and inflammatory and neurodegenerative disease states.
L G, Fröhlich   +9 more
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Distribution of 6R-L-Erythro-5,6,7,8-Tetrahydrobiopterin in Regional Brain Areas of Inbred Strains of Rats and Mice with Different Alcohol Preferences

Neuropsychobiology, 1996
6R-L-erythro-5,6,7,8-tetrahydrobiopterin (6R-BH4) is a coenzyme for tyrosine, tryptophan and phenylalanine hydroxylases, the former two of which are the initial and the rate-limiting enzymes in the biosynthesis of catecholamines and serotonin, respectively.
K, Yoshimoto   +6 more
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Enhancement of Dopamine Release In Vivo from the Rat Striatum by Dialytic Perfusion of 6Rlerythro‐5,6,7,8‐Tetrahydrobiopterin

Journal of Neurochemistry, 1990
Abstract:We have previously reported that intracerebroventricular administration of 6R‐L‐erythro‐5,6,7,8‐tetrahydrobiopterin (6R‐BH4), a cofactor for tyrosine hydroxylase, enhances biosynthesis of 3,4‐dihydroxyphenylethylamine (dopamine) in the rat brain. In the present study, we have more precisely examined the effects of 6R‐BH4 on dopamine release in
K, Koshimura   +4 more
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Effect of 6R-L-erythro-5,6,7,8-tetrahydrobiopterin on in vivo L-[?-11C]DOPA turnover in the rat striatum with infusion of L-tyrosine

Journal of Neural Transmission, 1994
L-[11C]DOPA, combined with positron emission tomography (PET), has made possible the assessment of dopamine turnover in vivo. Before the evaluation of PET study with L-[11C]DOPA in the primate, the effect of 6R-L-erythro-5,6,7,8-tetrahydrobiopterin (6R-BH4) and/or L-tyrosine infusion on L-[11C]DOPA turnover was analyzed in the rat striatal tissue and ...
H, Tsukada   +8 more
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Evidence for Specific Complex Formation between α-Melanocyte Stimulating Hormone and 6(R)-l-Erythro-5,6,7,8-tetrahydrobiopterin using Near Infrared Fourier Transform Raman Spectroscopy

Biochemistry, 1999
The cofactor 6(R)-L-erythro-5,6,7,8-tetrahydrobiopterin (6BH(4)) and its 2 and 4 electron oxidation products 7,8-dihydro-L-biopterin and L-biopterin have been shown to form 1:1 complexes with the thirteen amino acid peptide alpha-melanocyte stimulating hormone (alpha-MSH). Hydrogen bonding to the pyrimidine ring of the cofactor has been established for
J, Moore, J M, Wood, K U, Schallreuter
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Effect of 6R-l-erythro-5,6,7,8-tetrahydrobiopterin and infusion of l-tyrosine on the in vivo l-[β-11C]DOPA disposition in the monkey brain

Brain Research, 1996
The effect of 6R-L-erythro-5,6,7,8-tetrahydrobiopterin (6R-BH4) and L-tyrosine infusion on [11C]dopamine synthesis was analyzed in the striatum of Rhesus using positron emission tomography (PET). The rate for decarboxylation from L-[beta-11C]DOPA to [11C]dopamine was calculated using a graphical method with cerebellum as a reference region.
H, Tsukada   +9 more
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The Effects of 6R-L-Erythro-5,6,7,8-Tetrahydrobiopterin on Ethanol-Induced Sleep Time and Ethanol Elimination in Inbred Strains of Mice with Different Alcohol Preference

Neuropsychobiology, 1997
6R-L-erythro-5,6,7,8-tetrahydrobiopterin (6R-BH4) is a coenzyme for tyrosine, tryptophan and phenylalanine hydroxylases. Male C57BL/6J and DBA/2J mice were given 6R-BH4 (0, 12.5, 25.0 and 50.0 mg/kg of body weight, i.v.) or saline, and then 4 h later, all animals were injected with ethanol (EtOH) (4.0 g/kg, i.p.), which causes them to lose the righting
K, Yoshimoto   +3 more
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