Results 111 to 120 of about 20,824 (230)

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

Nitrite Generation in Interleukin-4—Treated Human Macrophage Cultures Does Not Involve the Nitric Oxide Synthase Pathway [PDF]

, 2017
The search continues for high-output nitric oxide biosynthesis in human macrophages analogous to murine phagocytes. Recently, generation of nitrite in culture supernatants of human macrophages exposed to interferon-γ and interleukin-4 (IFN-γ/IL-4) was ...
Blau, N., Linscheid, P., Schaffner, A., Schneemann, M., Schoedon, G., Walter, R.   +5 more
core  

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

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

Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo [PDF]

, 2017
The recent approval of sapropterin dihydrochloride, the synthetic form of 6[R]-l-erythro-5,6,7,8-tetrahydrobiopterin (BH4), for the treatment of phenylketonuria (PKU) as the first pharmacological chaperone drug initiated a paradigm change in the ...
Danecka, Marta K., Domdey, Katharina A., Eichinger, Anna, Fingerhut, Ralph, Gersting, Søren W., Glossmann, Hartmut, Kemter, Kristina F., Lagler, Florian B., Messing, Dunja D., Muntau, Ania C., Roscher, Adelbert A., Staudigl, Michael, Zsifkovits, Clemens   +12 more
core  

Nitric Oxide and Infection: Another View [PDF]

, 2017
Nitric oxide (NO) has been nicknamed "murderer” and "mediator” because it has toxic and signaling properties. We review these two aspects of NO synthesis from the perspective of the clinical infectious disease specialist by considering the potential of ...
Blan, N., Hofer, S., Schaffner, A., Schneemann, M., Schoedon, G., Walter, R.   +5 more
core  

Effects of Sapropterin on Endothelium-Dependent Vasodilation in Patients With CADASIL: A Randomized Controlled Trial [PDF]

Background and Purpose-Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a rare autosomal dominant disorder caused by NOTCH3 mutations, is characterized by vascular smooth muscle and endothelial cells ...
Campolo, Jonica, De Maria, Renata, Federico, Antonio, Frontali, Marina, Parodi, Oberdan, Parolini, Marina, Taroni, Franco   +6 more
core  

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

Effects of Granulocyte-Colony-Stimulating Factor in Neutropenic AIDS-Patients [PDF]

, 1992
Bogner, Johannes R., Goebel, Frank-Detlef, Heinrich, B., Kronawitter, U., Matuschke, A.   +4 more
core   +1 more source

Diagnosis of tetrahydrobiopterin deficiency using filter paper blood spots: further development of the method and 5 years experience [PDF]

, 2018
In every newborn with even mild hyperphenylalaninemia (HPA) tetrahydrobiopterin (BH4) deficiencies need to be excluded as soon as possible. Differential diagnosis is most commonly performed by analysis of urinary neopterin and biopterin.
Abu Seda, Bettina, Blau, Nenad, Hoffmann, Georg, Opladen, Thomas, Rassi, Anahita, Thöny, Beat   +5 more
core