Age-Dependent Variation in Blood Biopterin Peaks Following Oral Tetrahydrobiopterin Administration in Phenylketonuria. [PDF]
J Inherit Metab DisABSTRACT The correct diagnosis of tetrahydrobiopterin (BH4, sapropterin dihydrochloride)‐responsive phenylketonuria (PKU) and treatment with BH4 are important for prognosis and quality of life. We examined whether age affects biopterin bioavailability following oral BH4 administration in PKU and whether this influences BH4 responsiveness.
Kitayama K +5 more
europepmc +2 more sources
The Sooner, the Better: Neuroprotective Strategies in Fetuses With Congenital Heart Disease. [PDF]
Prenat DiagnABSTRACT Congenital heart disease (CHD) is the most frequent congenital malformation at birth and is associated with neurodevelopmental impairments. Alterations in cardiovascular physiology can lead to reduced cerebral blood perfusion and oxygenation, which negatively affects brain growth and maturation.
Nijman M +7 more
europepmc +2 more sources
TETRAHYDROBIOPTERIN BIOSYNTHESIS [PDF]
, 1982 published
Curtius, Hans-Christoph +3 more
openaire +2 more sources
Mutationen im PTS-Gen und mögliche Auswirkungen auf Funktion und Struktur der 6-Pyruvoyl-Tetrahydropterin-Synthase [PDF]
, 2002 Background: Research on Juvenile Idiopathic Arthritis (JIA) should support patients, caregivers/parents (carers) and clinicians to make important decisions in the consulting room and eventually to improve the lives of patients with JIA.
Preuße, Friedrich-Alexander +1 more
core +9 more sources
Tetrahydrobiopterin in biomedical research [PDF]
Journal of Inherited Metabolic Disease, 2009 Tetrahydrobiopterin (BH4) is an endogenously synthesized cofactor, required for various enzyme activities and for some less well-defined functions. It is also required at the cellular level. The known enzymes that depend on BH4 are phenylalanine hydroxylase (PAH), tyrosine hydroxylase, tryptophan hydroxylases 1 and 2, all three types of nitric oxide ...
Blau, Nenad, Thöny, Beat
openaire +4 more sources
A Comparison of Sepiapterin and Tetrahydrobiopterin Uptake by BRL2H3 Cells
Pteridines, 1996 Cellular uptake of sepiapterin and tetrahydrobiopterin was compared quantitatively with RBL2H3 cells in culture. RBL2H3 is a mast cell line and has the ability to synthesize serotonin.
Yamamoto Kazumasa +3 more
doaj +1 more source
Examining the role of insulin in the regulation of cardiovascular health [PDF]
, 2013 A substantial body of evidence has reported that insulin has direct actions on the cardiovascular system independent of its systemic effects on plasma glucose or lipids.
Salt, Ian P.
core +1 more source
The International Working Group on Neurotransmitter related Disorders (iNTD): A worldwide research project focused on primary and secondary neurotransmitter disorders [PDF]
, 2016 INTRODUCTION: Neurotransmitters are chemical messengers that enable communication between the neurons in the synaptic cleft. Inborn errors of neurotransmitter biosynthesis, breakdown and transport are a group of very rare neurometabolic diseases ...
Cortès-Saladelafont, Elisenda +17 more
core +4 more sources
Tetrahydrobiopterin in antenatal brain hypoxia-ischemia-induced motor impairments and cerebral palsy
Redox Biology, 2017 Antenatal brain hypoxia-ischemia, which occurs in cerebral palsy, is considered a significant cause of motor impairments in children. The mechanisms by which antenatal hypoxia-ischemia causes brain injury and motor deficits still need to be elucidated ...
Jeannette Vasquez-Vivar +4 more
doaj +1 more source
Pteridines, 2013 Ischemia-reperfusion injury is a primarily non-allospecific event leading to the depletion of the essential nitric oxide synthase cofactor and potent antioxidant tetrahydrobiopterin.
Cardini Benno +10 more
doaj +1 more source