First insights into structure-function relationships of alkylglycerol monooxygenase
Alkylglycerol monooxygenase is a tetrahydrobiopterin-dependent enzyme that cleaves the O-alkyl-bond of alkylglycerols. It is an exceptionally unstable, hydrophobic membrane protein which has never been purified in active form.
Watschinger Katrin+8 more
doaj +1 more source
GTPCH (GTP cyclohydrolase 1, encoded by Gch1) is required for the synthesis of tetrahydrobiopterin; a critical regulator of endothelial NO synthase function.
S. Chuaiphichai+8 more
semanticscholar +1 more source
Inherited metabolic epilepsies–established diseases, new approaches
Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
wiley +1 more source
Increasing Tetrahydrobiopterin in Cardiomyocytes Adversely Affects Cardiac Redox State and Mitochondrial Function Independently of Changes in NO Production [PDF]
Tetrahydrobiopterin (BH4) represents a potential strategy for the treatment of cardiac remodeling, fibrosis and/or diastolic dysfunction. The effects of oral treatment with BH4 (Sapropterin™ or Kuvan™) are however dose-limiting with high dose negating ...
Bennett, Brian+5 more
core +1 more source
Compensatory Feto-Placental Upregulation of the Nitric Oxide System during Fetal Growth Restriction [PDF]
Background: Fetal Growth Restriction is often associated with a feto-placental vascular dysfunction conceivably involving endothelial cells. Our study aimed to verify this pathogenic role for feto-placental endothelial cells and, coincidentally ...
Begliuomini, Silvia+11 more
core +3 more sources
Molecular genetics of tetrahydrobiopterin deficiency in Chinese patients
Background The overall incidence of hyperphenylalaninemia (HPA) in China is 1:11,763, with tetrahydrobiopterin (BH4) deficiency accounting for 8.55% of patients with HPA in the mainland.
Nana Li+12 more
semanticscholar +1 more source
The purpose of this study was to characterize the protective effect of tetrahydrobiopterin (BH4), one of the cofactors of nitric oxide (NO) synthase, against NO-induced endothelial cell injury. The addition of S-nitroso-N-acetyl-D,L-penicillamine (SNAP),
Ishii Masakazu+4 more
doaj +1 more source
Trends in resource utilization for new‐onset psychosis hospitalizations at children's hospitals
Abstract Background Children with new‐onset psychosis often require hospitalization for medical evaluation. Objectives The goal of this study was to assess variations in the management of children with new‐onset psychosis and characterize trends in resource utilization.
Ankita Gupta+8 more
wiley +1 more source
Modulation of interleukin 2 high affinity binding to human T cells by a pyrimidodiazepine insect metabolite [PDF]
An insect metabolite containing the little known pyrimido[4,5-b][l,4]diazepine ring system has been found to act as an effective mimic of tetrahydrobiopterin in its ability to modulate the affinity of interleukin 2 (IL-2) for its receptors on human T ...
Borchert, Monika+4 more
core +1 more source
Insulin Stimulates Tetrahydrobiopterin Synthesis in Mouse Brain Microvascular Endothelial Cells
We examined the effects of insulin on tetrahydrobiopterin (BH4) synthesis in mouse brain microvascular endothelial cells (MBMECs). Treatment of MBMECs with insulin increased the intracellular BH4 content in a time- and concentration-dependent manner. The
Ishi Masakazu+4 more
doaj +1 more source