Results 111 to 120 of about 20,671 (210)

Influences of Catecholamine Contents on Tetrahydrobiopterin Metabolism

Pteridines, 1998
The influence of changes in catecholamine metabolism on tetrahydrobiopterin biosynthesis was investigated in cultured rat pheochromocytoma PC12 cells. The increase in the cellular dopamine content after treatment with the MAO-COMT inhibitor or dopamine ...
Kuzuya Hiroshi, Sakamoto Hideo, Fujita Ko   +2 more
doaj   +1 more source

Dysregulated Choline, Methionine, and Aromatic Amino Acid Metabolism in Patients with Wilson Disease: Exploratory Metabolomic Profiling and Implications for Hepatic and Neurologic Phenotypes. [PDF]

, 2019
Wilson disease (WD) is a genetic copper overload condition characterized by hepatic and neuropsychiatric symptoms with a not well-understood pathogenesis.
Czlonkowska, Anna, Kim, Kyoungmi, Litwin, Tomasz, Mazi, Tagreed A, Medici, Valentina, Sarode, Gaurav V, Shibata, Noreene M   +6 more
core   +2 more sources

Pterin Metabolism In Haemodialysis Patients

Pteridines, 1989
Patients undertaking maintenance haemodialysis may develop a variety of neurological disorders, the most serious of which is aluminium induced encephalopathy.
Altmann Paul, Sawyer Nick, Cunningham John, Marsh Frank, Hamon Chris, Blair John A.   +5 more
doaj   +1 more source

Influence of tetrahydrobiopterin supplementation on rate pressure product [PDF]

, 2015
Augmentation of tetrahydrobiopterin (BH4) could potentially improve eNOS uncoupling by increasing Nitric oxide (NO) bioavailability to improve endothelial health in cardiovascular diseases.
Barlow, Matthew, Dr, Cheeti, Upender, Proctor, David, Dr   +2 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

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  

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

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  

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
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