Tetrahydrobiopterin biosynthesis, regeneration and functions [PDF]
Biochemical Journal, 2000 Tetrahydrobiopterin (BH4) cofactor is essential for various processes, and is present in probably every cell or tissue of higher organisms. BH4 is required for various enzyme activities, and for less defined functions at the cellular level.
Nenad Blau +2 more
semanticscholar +5 more sources
Novel perspectives in redox biology and pathophysiology of failing myocytes: modulation of the intramyocardial redox milieu for therapeutic interventions - A review article from the Working Group of Cardiac Cell Biology, Italian Society of Cardiology [PDF]
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2016 The prevalence of heart failure (HF) is still increasing worldwide, with enormous human, social, and economic costs, in spite of huge efforts in understanding pathogeneticmechanisms and in developing effective therapies that have transformed this ...
Angelini, Annalisa +8 more
core +15 more sources
A Comparison of Sepiapterin and Tetrahydrobiopterin Uptake by BRL2H3 Cells [PDF]
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 +2 more sources
The auto‐oxidation of tetrahydrobiopterin [PDF]
European Journal of Biochemistry, 1988 The product of the aerobic oxidation of tetrahydrobiopterin, quinonoid dihydrobiopterin, is unstable and rapidly rearranges to form a 7,8‐dihydropteridine. Kaufman [Kaufman, S. (1967) J. Biol. Chem. 242, 3934–3943] identified the stable product produced in 0.1 M phosphate pH 6.8, as 7,8‐dihydrobiopterin. However, Armarego et al. [Armarego, W. L.
Seymour Kaufman +2 more
openaire +4 more sources
Tetrahydrobiopterin Biosynthesis in Man [PDF]
, 1986 published
Curtius, Hans-Christoph +3 more
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Tetrahydrobiopterin Loading Test in Hyperphenylalaninemia [PDF]
Pediatric Research, 1991 Some cases of primary hyperphenylalaninemia are not caused by the lack of phenylalanine hydroxylase, but by the lack of its cofactor tetrahydrobiopterin. These patients are not clinically responsive to a phenylalanine-restricted diet, but need specific substitution therapy. Thus, it became necessary to examine all newborns screened as positive with the
PONZONE, Alberto +5 more
openaire +5 more sources
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-carbinolamine dehydratase.
Blau N, Thony B, Spada M, Ponzone A
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Tetrahydrobiopterin protects phenylalanine hydroxylase activity in vivo: Implications for tetrahydrobiopterin-responsive hyperphenylalaninemia [PDF]
FEBS Letters, 2004 The natural cofactor of phenylalanine hydroxylase (PAH), tetrahydrobiopterin (BH4), regulates the enzyme activity as well as being essential in catalysis. BH4-responsive PAH deficiency is a variant of hyperphenylalaninemia or phenylketonuria (PKU) caused
Ding, Zhaobing +2 more
core +4 more sources
Monitoring Treatment in Tetrahydrobiopterin Deficiency [PDF]
pteridines, 1991 Spada M +6 more
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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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