Results 111 to 120 of about 23,011 (262)

Maintenance of cellular tetrahydrobiopterin homeostasis

BMB Reports, 2010
Tetrahydrobiopterin (BH4) is a multifunctional cofactor of aromatic amino acid hydroxylases and nitric oxide synthase (NOS) as well as an intracellular antioxidant in animals. Through regulation of NOS activity BH4 plays a pivotal role not only in a variety of normal cellular functions but also in the pathogenesis of cardiovascular and ...
Hye-Lim, Kim, Young Shik, Park
openaire   +3 more sources

Long-Term Treatment and Diagnosis of Tetrahydrobiopterin-Responsive Hyperphenylalaninemia with a Mutant Phenylalanine Hydroxylase Gene [PDF]

, 2003
Haruo Shintaku, Shigeo Kure, Toshihiro Ohura, Yoshiyuki Okano, Misao Ohwada, Naruji Sugiyama, Nobuo Sakura, Ichiro Yoshida, Makoto Yoshino, Yoichi Matsubara, Ken Suzuki, Kikumaro Aoki, Teruo Kitagawa   +12 more
openalex   +1 more source

Native and Phosphorylated Bovine Adrenal Tyrosine 3-Monooxygenase. Interactions with Tetrahydropterins and Substrate and Stability of the Formed 4a-Hydroxy-Tetrahydrobiopterin

Pteridines, 1989
The catalytic activity of tyrosine 3-monooxygenase (tyrosine hydroxylase) is dependent on a tetrahydropterin cofactor and ezyme-bound iron. The oxidation of tetrahydrobiopterin by bovine adrenal tyrosine hydroxylase was studied by high performance liquid
Haavik Jan, Andersson Kristoffer K., Flatmark Torgeir, Petersson Leif   +3 more
doaj   +1 more source

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

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  

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

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

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

Critical Role for Tetrahydrobiopterin Recycling by Dihydrofolate Reductase in Regulation of Endothelial Nitric-oxide Synthase Coupling [PDF]

, 2009
Mark J. Crabtree, Amy L. Tatham, Ashley Hale, Nicholas J. C. King, Keith M. Channon   +4 more
openalex   +1 more source

Analgesic Effect of Tranilast in an Animal Model of Neuropathic Pain and Its Role in the Regulation of Tetrahydrobiopterin Synthesis [PDF]

, 2022
Swarnalakshmi Raman, Arief Waskitho, Resmi Raju, Takuma Iwasa, Daisuke Ikutame, Kazuo Okura, Masamitsu Oshima, Yoshizo Matsuka   +7 more
openalex   +1 more source