Results 101 to 110 of about 14,039 (215)

Tetrahydrobiopterin deficiencies in the Maltese population [PDF]

, 2002
A higher than usual frequency of hyperphenylalaninaemia due to tetrahydrobiopterin (BH4) deficiencies, specifically Dihydropteridine Reductase (DHPR) deficiency, is present in the Maltese population.
34th ESHG Conference, Naudi, R., Scerri, Christian A., Felice, A. E., Farrugia, Rosienne, Attard Montalto, S., Bartolo, C., Parascandolo, Raymond   +7 more
core  

Glucocorticoids Inhibit Tetrahydrobiopterin-Dependent Endothelial Function

, 2001
Tetrahydrobiopterin (BH4) acts as an important co-factor for endothelial nitric oxide synthase (eNOS). Glucocorticoids have been shown to inhibit expression of the rate-limiting enzyme for tetrahydrobiopterin synthesis, GTP cyclohydrolase, in other cell
Douglas G. Johns, Anne M. Dorrance, R. Clinton Webb, Nicole L. Tramontini   +3 more
core   +1 more source

6R-tetrahydrobiopterin treated PKU patients below 4years of age: Physical outcomes, nutrition and genotype

, 2015
Background and Aims Phenylalanine-restricted diets have proven effective in treating phenylketonuria. However, such diets have occasionally been reported to hinder normal development.
González-Lamuño, Domingo, Bueno, María A., Llarena, Marta, Couce, María L., García, María C., Lage, Sergio, Dalmau, Jaime, Ruiz, María A., Andrade, Fernando, Aldámiz-Echevarría, Luis, Blasco, Javier, González, David, Peña-Quintana, Luis, Vitoria, Isidro, Sánchez-Valverde, Felix, Ruiz, Mónica, Alcalde, Carlos, Gil, David   +17 more
core   +1 more source

Statin Increases GTP Cyclohydrolase I mRNA and 5,6,7,8-Tetrahydrobiopterin in Vascular Endothelial Cells

Pteridines, 2006
We investigated the effects of HMG-CoA reductase inhibitors, so-called statins, on 5,6,7,8-tctrahydrobioptcrin (BH4) metabolism in human umbilical vein endothelial cells (HUVEC).
Hattori Yoshiyuki, Nakanishi Nobuo
doaj   +1 more source

Tetrahydrobiopterin, its mode of action on phenylalanine hydroxylase, and importance of genotypes for pharmacological therapy of phenylketonuria

, 2013
In about 20%-30% of phenylketonuria (PKU) patients (all phenotypes of PAH deficiency), Phe levels may be controlled through phenylalanine hydroxylase cofactor tetrahydrobiopterin therapy.
Blau, Nenad, Heintz, Caroline, Cotton, Richard G H   +2 more
core   +1 more source

Immune activation and inflammation in patients with cardiovascular disease are associated with elevated phenylalanine-to-tyrosine ratios

Pteridines, 2013
Higher serum neopterin concentrations and kynurenine-to-tryptophan (Kyn/Trp) ratios are associated with increased mortality in patients with coronary artery disease (CAD). Preferentially, Th1-type cytokine interferon-γ stimulates tryptophan breakdown and
Mangge Harald, Schnedl Wolfgang J., Schröcksnadel Sebastian, Geisler Simon, Murr Christian, Fuchs Dietmar   +5 more
doaj   +1 more source

Tetrahydrobiopterin precursor sepiapterin provides protection against neurotoxicity of 1-methyl-4-phenylpyridinium in nigral slice cultures

, 2003
Udgivelsesdato: 2003-AprComplex-I inhibition and oxidative processes have been implicated in the loss of nigral dopamine neurones in Parkinson's disease and the toxicity of MPTP and its metabolite MPP+.
Gramsbergen, Jan Bert, Morten Meyer, Zimmer, Jens, Meyer, Morten, Jakob Torp Madsen, Christian Hesslinger, Jens Zimmer, Madsen, Jakob Torp, Jan Bert Gramsbergen, Hesslinger, Christian, Pernille Jansen, Jansen, Pernille   +11 more
core   +1 more source

Maximal dietary responsiveness after tetrahydrobiopterin (BH4) in 19 phenylalanine hydroxylase deficiency patients: What super-responders can expect

Molecular Genetics and Metabolism Reports
Background: Inherited phenylalanine hydroxylase deficiency, also known as phenylketonuria (PKU), causes poor growth and neurologic deficits in the untreated state.
Jariya Upadia, Kea Crivelly, Grace Noh, Amy Cunningham, Caroline Cerminaro, Yuwen Li, Meredith Mckoin, Madeline Chenevert, Hans C. Andersson   +8 more
doaj   +1 more source

A Phase 1 Study to Assess the Pharmacokinetics, Food Effect, Safety, and Tolerability of Sepiapterin in Healthy Japanese and Non-Japanese Participants

Pharmaceuticals
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, Diksha Kaushik, Kimberly Ingalls, Neil Smith, Ronald Kong   +4 more
doaj   +1 more source

Delineation of the arginine- and tetrahydrobiopterin-binding sites of neuronal nitric oxide synthase.

, 1997
Nitric oxide synthase (EC 1.14.13.39) catalyses the conversion of arginine, NADPH and oxygen to nitric oxide and citrulline, using haem, (6R)-5,6,7,8-tetrahydro-l-biopterin (tetrahydrobiopterin), calmodulin, FAD and FMN as cofactors.
Lowe, PN, Smith, D, Saqi, M, Boyhan, A, Charles, IG   +4 more
core   +1 more source