Results 11 to 20 of about 14,039 (215)
Tetrahydrobiopterin and inherited hyperphenylalaninemias
The Turkish Journal of Pediatrics, 1996 Tetrahydrobiopterin deficiency, a variant of hyperphenylalaninemia, may be caused by deficiency of one of the following enzymes: guanosine triphosphate cyclohydrolase 1,6-pyruvoyltetrahydropterin synthase, dihydropteridin reductase and pterin-4a ...
N Blau, B Thony, M Spada, A Ponzone
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Tetrahydrobiopterin Deficiency: From Phenotype to Genotype [PDF]
Pteridines, 1993 As a result of the selective screening worldwide during the last 18 years, approximately 250 patients with tetrahydrobiopterin deficiency were discovered.
Blau Nenad +3 more
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Three classes of tetrahydrobiopterin-dependent enzymes
Pteridines, 2013 Current knowledge distinguishes three classes of tetrahydrobiopterin-dependent enzymes as based on protein sequence similarity. These three protein sequence clusters hydroxylate three types of substrate atoms and use three different forms of iron for ...
Werner Ernst R.
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Tetrahydrobiopterin analogues with NO-dependent pulmonary vasodilator properties [PDF]
, 2011 Reduced NO levels due to the deficiency of tetrahydrobiopterin (BH4) contribute to impaired vasodilation in pulmonary hypertension Due to the chemically unstable nature of BH4 it was hypothesised that oxidatively stable analogues of BR, would be able to ...
Roger M. Wadsworth +9 more
core +4 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
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TETRAHYDROBIOPTERIN BIOSYNTHESIS [PDF]
, 1982 published
Curtius, Hans-Christoph +3 more
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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
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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
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Response of Phenylketonuria to Tetrahydrobiopterin [PDF]
The Journal of Nutrition, 2007 A favorable response, indicated by decline of blood phenylalanine (Phe) in patients with phenylketonuria (PKU), to orally administered 6-R-L-erythro-5, 6, 7, 8-tetrahydrobiopterin (BH4) has been reported in many countries following the first publication in 1999. In this review, we describe the experience in the United States with PKU patients and their
Kimberlee, Michals-Matalon +4 more
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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
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