BH4, tetrahydrobiopterin
Cuticle Integrity and Biogenic Amine Synthesis in Caenorhabditis elegans Require the Cofactor Tetrahydrobiopterin (BH4)
... ABSTRACT Tetrahydrobiopterin (BH4) is the natural cofactor of several enzymes widely distributed among eukaryotes, including aromatic amino acid hydroxylases (AAAHs), nitric oxide synthases (NOSs), and ...
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One-year follow-up of B vitamin and Iron status in patients with phenylketonuria provided tetrahydrobiopterin (BH4)
... to tetrahydrobiopterin (BH4), an enzyme cofactor necessary for the catalysis of phe to tyrosine (tyr) by phenylalanine hydroxylase (PAH), may benefit from BH4-driven therapy such as sapropterin ...
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Tetrahydrobiopterin (BH4) deficiency is associated with augmented inflammation and microvascular degeneration in the retina
... 1 Tetrahydrobiopterin (BH4) deficiency is associated with morphological changes in the ...c BH4 content (ng/mg of tissue) in retinas from control (WT) and hph-1 mice at P22 was measured by using ...
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Biopterin synthesis defect Treatment with L dopa and 5 hydroxytryptophan compared with therapy with a tetrahydropterin
... Abbreviations used in this paper: BH4, tetrahydrobiopterin; CSF, cerebrospinal fluid; DA, dopamine; DHPG, 3,4-dihydroxyphenylethyleneglycol; DHPR, dihydropteridine reductase; DOPAC, dihy[r] ...
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Mechanisms of endothelial dysfunction in rheumatoid arthritis: lessons from animal studies
... Abbreviations ACE: Angiotensin-converting enzyme; ACh: Acetylcholine; AIA: Adjuvantinduced arthritis; ANG-II: Angiotensin II; ARB: Angiotensin receptor blocker; BH4: Tetrahydrobiopterin;[r] ...
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Tetrahydrobiopterin restores endothelial function in hypercholesterolemia
... of tetrahydrobiopterin (BH4), an essential cofactor of NOS (14, 15), uncoupling of the l -arginine–NO pathway is observed, which results in increased formation of oxygen radicals by NOS in vitro (15, ...
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Tetrahydrobiopterin, L-Arginine and Vitamin C Act Synergistically to Decrease Oxidant Stress and Increase Nitric Oxide That Increases Blood Flow Recovery after Hindlimb Ischemia in the Rat
... L -arginine (6 months) not only failed to demonstrate a beneficial effect, but re- sulted in a degree of eNOS-dependent vascular reactivity significantly less than that of the placebo group (16). The pres- ent findings ...
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Early Screening for Tetrahydrobiopterin Responsiveness in Phenylketonuria
... of BH4 therapy ranges from $10 000 to more than $80 000 per patient per year, depending on dose and ...as BH4 responsive. The current definition of BH4 responsiveness is based on the decrease of ...
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Long-term follow-up of patients with phenylketonuria treated with tetrahydrobiopterin: a seven years experience
... with BH4 at the initial dose of 10 ...mg BH4 tablets (Schircks Laboratories), divided in three oral doses before meals, until September 2009, when therapy was shifted to ...
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Is tetrahydrobiopterin a therapeutic option in diabetic hypertensive patients?
... Because folic acid participates in the BH4 recycling pathway, several studies have been performed to investigate its use for the treatment of cardiovascular disease. Some results had shown that this agent may ...
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Tetrahydrobiopterin analogues with NO-dependent pulmonary vasodilator properties
... of BH4 with DAHP, and treatment with ADDP did not signi fi cantly change the carbachol concentration – response ...eliminated BH4 from the vascular ...
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Differential Diagnosis of Variant Forms of Hyperphenylalaninemia
... Another of the newer methods involves measure- ment of the patient’s blood phenylalanine levels after the administration of tetrahydrobiopterin (BH4); phenylalanine levels decrease in pa[r] ...
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The neonatal tetrahydrobiopterin loading test in phenylketonuria: what is the predictive value?
... the BH4 loading test at neonatal age in predicting long-term BH4 responsiveness is that at neonatal age Phe concentration is heavily influenced by the catabolic or anabolic state of the ...predict ...
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Methylglyoxal modulates endothelial nitric oxide synthase-associated functions in EA.hy926 endothelial cells
... as BH4 but, unlike BH4, is ineffective in restoring uncoupled eNOS ...for BH4 synthesis via the pterin salvage pathway, was used to increase cellular BH4 levels ...
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Tetrahydrobiopterin responsiveness in phenylketonuria: prediction with the 48-hour loading test and genotype
... the BH4 intro- duction, all known values from the year preceding the BH4 loading test were used, with a minimum of four measurements; alternatively, the period was extended to two years or this value was ...
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BCL2-BH4 antagonist BDA-366 suppresses human myeloma growth
... a BH4-based BCL2 inhibitor to treat MM is distinct from this BH3 mimetics strategy, since BDA-366 not only blocks the activities of the BH4 domain, but also changes the conformation of BCL2 in the ...
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A crustacean nitric oxide synthase expressed in nerve ganglia, Y organ, gill and gonad of the tropical land crab, Gecarcinus lateralis
... Fig.·2. Comparison of deduced amino acid sequences of NO synthase from land crab, insects, mollusk and human. Land crab (Gecarcinus lateralis) NOS was aligned with NOS sequences from insects (Manduca sexta, Bombyx mori, ...
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Tetrahydrobiopterin alters superoxide and nitric oxide release in prehypertensive rats
... The A23187-stimulated production of O2- and its superoxide dismutase product hydrogen peroxide were significantly higher, whereas nitric oxide release was reduced in SHR aortas, with opp[r] ...
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Overexpression of endothelial nitric oxide synthase accelerates atherosclerotic lesion formation in apoE deficient mice
... with tetrahydrobiopterin, an NOS cofactor, reduced the atherosclerotic lesion size in apoE-KO/eNOS-Tg mice to the level com- parable to apoE-KO mice, possibly through the improvement of eNOS ...
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Regulation of nitric oxide synthesis by proinflammatory cytokines in human umbilical vein endothelial cells Elevations in tetrahydrobiopterin levels enhance endothelial nitric oxide synthase specific activity
... Regulation of Nitric Oxide Synthesis by Proinflammatory Cytokines in Human Umbilical Vein Endothelial Cells Elevations in Tetrahydrobiopterin Levels Enhance Endothelial Nitric Oxide Synt[r] ...
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