Results 271 to 280 of about 26,569 (299)
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Tetrahydrobiopterin regulation of eNOS redox function.
Current pharmaceutical design, 2014Tetrahydrobiopterin (BH4) is an essential cofactor of nitric oxide synthase (NOS). In the cardiovascular system, endothelial NOS (eNOS) has a major role in maintaining vascular tone and endothelial function, as well as in mediating many other vascular ...
Dan-Dan Chen +7 more
semanticscholar +1 more source
International database of tetrahydrobiopterin deficiencies
Journal of Inherited Metabolic Disease, 1995SummaryApproximately 2% of newborns with hyperphenylalaninaemia are deficient in tetrahydrobiopterin. Selective screening must be performed in all instances where hyperphenylalaninaemia is detected by neonatal screening. In the last 20 years, 308 patients with tetrahydrobiopterin deficiencies have been recognized as a result of screening carried out ...
Nenad Blau, I. Barnes, J. L. Dhondt
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Molecular and metabolic bases of tetrahydrobiopterin (BH4) deficiencies.
Molecular Genetics and Metabolism, 2021N. Himmelreich, N. Blau, B. Thöny
semanticscholar +1 more source
Classifying tetrahydrobiopterin responsiveness in the hyperphenylalaninaemias
Journal of Inherited Metabolic Disease, 2008SummaryBackgroundA significant percentage of patients with hyperphenylalaninaemia (HPA) due to primary deficiency of the phenylalanine hydroxylase enzyme (PAH) respond to a dose of tetrahydrobiopterin (BH4) with an increased rate of phenylalanine (Phe) disposal. The effect is exploited therapeutically, with some patients on BH4 even tolerating a normal
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Journal of child and adolescent psychopharmacology, 2013
OBJECTIVE The purpose of this study was to determine if tetrahydrobiopterin (BH4) reduced core symptoms of autism spectrum disorder (ASD). METHOD In this study, 46 children, 3-7 years of age diagnosed with an ASD were randomly assigned to double-blind ...
C. Klaiman +3 more
semanticscholar +1 more source
OBJECTIVE The purpose of this study was to determine if tetrahydrobiopterin (BH4) reduced core symptoms of autism spectrum disorder (ASD). METHOD In this study, 46 children, 3-7 years of age diagnosed with an ASD were randomly assigned to double-blind ...
C. Klaiman +3 more
semanticscholar +1 more source
Regulation of Tetrahydrobiopterin Biosynthesis
2002Tetrahydrobiopterin (6R-(L-erythro-dihdroxypropyl)-5,6,7,8-tetrahydropterin; BH4) is required as an essential intracellular cofactor of aromatic amino acid hydroxylases in the formation of catecholamines. This compound is a very unstable substance having a very fast turnover in vivo, and its tissue level is controlled at least by the rate of de novo ...
Yasumitsu Sakamoto +4 more
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Tetrahydrobiopterin as Another EDRF in Man
Biochemical and Biophysical Research Communications, 1994Endotoxin and inflammatory cytokines downregulate expression of constitutive nitric oxide synthase (cNOS) in human vascular endothelial cells with concomitant increase of tetrahydrobiopterin synthesis in these cells and parallel upregulation of inducible NOS expression in smooth muscle cells, indicating compartmentalized nitric oxide (NO) production ...
J. Steurer +5 more
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Tetrahydrobiopterin and Nitric Oxide Synthase Recouplers.
Handbook of Experimental Pharmacology, 2020K. Channon
semanticscholar +1 more source
Tetrahydrobiopterin and phenylketonuria
The Journal of Pediatrics, 2011PORTA, FRANCESCO +4 more
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Tetrahydrobiopterin in energy metabolism and metabolic diseases.
Pharmacological Research, 2020Hyoung-Kyu Kim, Jin Han
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