1421-63-2 | 2,4,5-Trihydroxybutyrophenone

Packsize	Purity	Availability	Price	Discounted Price
250mg	95%	in stock	$106.00	$75.00
1g	95%	in stock	$251.00	$176.00
5g	95%	in stock	$713.00	$499.00

Description

• Catalog Number: AA69535
• Chemical Name: 2,4,5-Trihydroxybutyrophenone
• CAS Number: 1421-63-2
• Molecular Formula: C10H12O4
• Molecular Weight: 196.1999
• MDL Number: MFCD00020019
• SMILES: CCCC(=O)c1cc(O)c(cc1O)O
• NSC Number: 73478

Computed Properties

• Complexity: 204
• Covalently-Bonded Unit Count: 1
• Heavy Atom Count: 14
• Hydrogen Bond Acceptor Count: 4
• Hydrogen Bond Donor Count: 3
• Rotatable Bond Count: 3
• XLogP3: 1.9

Literature

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• Erythropoietin prevents endothelial dysfunction in GTP-cyclohydrolase I-deficient hph1 mice.

  Journal of cardiovascular pharmacology 20141201

• Erythropoietin increases bioavailability of tetrahydrobiopterin and protects cerebral microvasculature against oxidative stress induced by eNOS uncoupling.

  Journal of neurochemistry 20141101

• α-Synuclein and mitochondrial bioenergetics regulate tetrahydrobiopterin levels in a human dopaminergic model of Parkinson disease.

  Free radical biology & medicine 20140201

• Resveratrol improves vascular function in patients with hypertension and dyslipidemia by modulating NO metabolism.

  Hypertension (Dallas, Tex. : 1979) 20130801

• Activation of PPARδ prevents endothelial dysfunction induced by overexpression of amyloid-β precursor protein.

  Cardiovascular research 20121201

• Cloning, expression and enzymatic properties analysis of dihydrofolate reductase gene from the silkworm, Bombyx mori.

  Molecular biology reports 20121201

• Simultaneous quantification of tetrahydrobiopterin, dihydrobiopterin, and biopterin by liquid chromatography coupled electrospray tandem mass spectrometry.

  Analytical biochemistry 20121115

• PPARδ agonist GW501516 prevents uncoupling of endothelial nitric oxide synthase in cerebral microvessels of hph-1 mice.

  Brain research 20121105

• Efficacy and safety of BH4 before the age of 4 years in patients with mild phenylketonuria.

  Journal of inherited metabolic disease 20121101

• Tetrahydrobiopterin (BH4) in PKU: effect on dietary treatment, metabolic control, and quality of life.

  Journal of inherited metabolic disease 20121101

• An international survey of patients with tetrahydrobiopterin deficiencies presenting with hyperphenylalaninaemia.

  Journal of inherited metabolic disease 20121101

• A volumetric study of basal ganglia structures in individuals with early-treated phenylketonuria.

  Molecular genetics and metabolism 20121101

• The role of tetrahydrobiopterin in inflammation and cardiovascular disease.

  Thrombosis and haemostasis 20121101

• Long-term treatment with tetrahydrobiopterin in phenylketonuria: treatment strategies and prediction of long-term responders.

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• Increased superoxide and endothelial NO synthase uncoupling in blood vessels of Bmal1-knockout mice.

  Circulation research 20121012

• The mechanism of BH4 -responsive hyperphenylalaninemia--as it occurs in the ENU1/2 genetic mouse model.

  Human mutation 20121001

• Tetrahydrobiopterin supplementation enhances carotid artery compliance in healthy older men: a pilot study.

  American journal of hypertension 20121001

• Prevention of lethal murine pancreas ischemia reperfusion injury is specific for tetrahydrobiopterin.

  Transplant international : official journal of the European Society for Organ Transplantation 20121001

• Administration of tetrahydrobiopterin improves the microcirculation and outcome in an ovine model of septic shock.

  Critical care medicine 20121001

• Tetrahydrobiopterin (BH4) in septic shock: a key to solving the nitric oxide puzzle and opening the microcirculation?

  Critical care medicine 20121001

• Association between the levels of circulating adhesion molecules and biopterins in type-2 diabetic normotensive patients adhesion molecules and biopterins.

  Endocrine, metabolic & immune disorders drug targets 20120901

• Tetrahydrobiopterin protects soluble guanylate cyclase against oxidative inactivation.

  Molecular pharmacology 20120901

• Recoupling the cardiac nitric oxide synthases: tetrahydrobiopterin synthesis and recycling.

  Current heart failure reports 20120901

• Uncoupling of eNOS causes superoxide anion production and impairs NO signaling in the cerebral microvessels of hph-1 mice.

  Journal of neurochemistry 20120901

• Utility of phenylalanine hydroxylase genotype for tetrahydrobiopterin responsiveness classification in patients with phenylketonuria.

  Molecular genetics and metabolism 20120901

• Assessment of tetrahydrobiopterin (BH(4))-responsiveness and spontaneous phenylalanine reduction in a phenylalanine hydroxylase deficiency population.

  Molecular genetics and metabolism 20120901

• Cardiomyocyte GTP cyclohydrolase 1 and tetrahydrobiopterin increase NOS1 activity and accelerate myocardial relaxation.

  Circulation research 20120831

• Tetrahydrobiopterin ameliorates hepatic ischemia-reperfusion Injury by coupling with eNOS in mice.

  The Journal of surgical research 20120801

• Preserving mitochondrial function prevents the proteasomal degradation of GTP cyclohydrolase I.

  Free radical biology & medicine 20120715

• Electron paramagnetic resonance characterization of tetrahydrobiopterin radical formation in bacterial nitric oxide synthase compared to mammalian nitric oxide synthase.

  Biophysical journal 20120703

• Recommendations for the use of sapropterin in phenylketonuria.

  Molecular genetics and metabolism 20120701

• Uncoupling endothelial nitric oxide synthase is ameliorated by green tea in experimental diabetes by re-establishing tetrahydrobiopterin levels.

  Diabetes 20120701

• Positive effect of a simplified diet on blood phenylalanine control in different phenylketonuria variants, characterized by newborn BH4 loading test and PAH analysis.

  Molecular genetics and metabolism 20120701

• Endothelial nitric oxide synthase impairment is restored by clofibrate treatment in an animal model of hypertension.

  European journal of pharmacology 20120615

• Methotrexate increases expression of cell cycle checkpoint genes via JNK activation.

  Arthritis and rheumatism 20120601

• Amelioration of renal injury and oxidative stress by the nNOS inhibitor L-VNIO in the salt-sensitive mRen2.Lewis congenic rat.

  Journal of cardiovascular pharmacology 20120601

• Endothelium-specific sepiapterin reductase deficiency in DOCA-salt hypertension.

  American journal of physiology. Heart and circulatory physiology 20120601

• Five novel mutations and two large deletions in a population analysis of the phenylalanine hydroxylase gene.

  Molecular genetics and metabolism 20120601

• [Risk-sharing scheme in Israel--Kuvan as an allegory].

  Harefuah 20120601

• Advanced glycation end products-mediated hypertrophy is negatively regulated by tetrahydrobiopterin in renal tubular cells.

  Molecular and cellular endocrinology 20120515

• Ascorbate stimulates endothelial nitric oxide synthase enzyme activity by rapid modulation of its phosphorylation status.

  Free radical biology & medicine 20120515

• Insulin-induced generation of reactive oxygen species and uncoupling of nitric oxide synthase underlie the cerebrovascular insulin resistance in obese rats.

  Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism 20120501

• The spectrum of mutations identified in Cypriot patients with phenylalanine hydroxylase deficiency detected through neonatal screening.

  Clinical biochemistry 20120501

• Novel pharmacological chaperones that correct phenylketonuria in mice.

  Human molecular genetics 20120415

• Fluvastatin upregulates endothelial nitric oxide synthase activity via enhancement of its phosphorylation and expression and via an increase in tetrahydrobiopterin in vascular endothelial cells.

  International journal of cardiology 20120405

• Nitric oxide synthases: regulation and function.

  European heart journal 20120401

• Catalytic residues and a predicted structure of tetrahydrobiopterin-dependent alkylglycerol mono-oxygenase.

  The Biochemical journal 20120401

• HPLC analysis of tetrahydrobiopterin and its pteridine derivatives using sequential electrochemical and fluorimetric detection: application to tetrahydrobiopterin autoxidation and chemical oxidation.

  Archives of biochemistry and biophysics 20120401

• Quantification of phenylalanine hydroxylase activity by isotope-dilution liquid chromatography-electrospray ionization tandem mass spectrometry.

  Molecular genetics and metabolism 20120401

• Evolving patient selection and clinical benefit criteria for sapropterin dihydrochloride (Kuvan®) treatment of PKU patients.

  Molecular genetics and metabolism 20120401

• Rapid clearance of supplemented tetrahydrobiopterin is driven by high-capacity transporters in the kidney.

  Molecular genetics and metabolism 20120401

• Which neuroprotective agents are ready for bench to bedside translation in the newborn infant?

  The Journal of pediatrics 20120401

• Dopamine agonists in dihydropteridine reductase deficiency.

  Molecular genetics and metabolism 20120401

• Direct detection of tetrahydrobiopterin (BH4) and dopamine in rat brain using liquid chromatography coupled electrospray tandem mass spectrometry.

  Biochemical and biophysical research communications 20120323

• Systemic and vascular oxidation limits the efficacy of oral tetrahydrobiopterin treatment in patients with coronary artery disease.

  Circulation 20120320

• Apocynin improves oxygenation and increases eNOS in persistent pulmonary hypertension of the newborn.

  American journal of physiology. Lung cellular and molecular physiology 20120315

• Regulation of β-adrenergic control of heart rate by GTP-cyclohydrolase 1 (GCH1) and tetrahydrobiopterin.

  Cardiovascular research 20120315

• [Treatment and control of patients with phenylketonuria: results from the Collaborative Group of Spanish Follow-up Units].

  Medicina clinica 20120303

• Local tetrahydrobiopterin administration augments reflex cutaneous vasodilation through nitric oxide-dependent mechanisms in aged human skin.

  Journal of applied physiology (Bethesda, Md. : 1985) 20120301

• Arg375 tunes tetrahydrobiopterin functions and modulates catalysis by inducible nitric oxide synthase.

  Journal of inorganic biochemistry 20120301

• Tetrahydrobiopterin improves endothelial function and decreases arterial stiffness in estrogen-deficient postmenopausal women.

  American journal of physiology. Heart and circulatory physiology 20120301

• Simvastatin ameliorates angiotensin II-induced endothelial dysfunction through restoration of Rho-BH4-eNOS-NO pathway.

  Cardiovascular drugs and therapy 20120201

• START, a double blind, placebo-controlled pharmacogenetic test of responsiveness to sapropterin dihydrochloride in phenylketonuria patients.

  Molecular genetics and metabolism 20120201

• Changes in eNOS phosphorylation contribute to increased arteriolar NO release during juvenile growth.

  American journal of physiology. Heart and circulatory physiology 20120201

• Propofol protects against high glucose-induced endothelial dysfunction in human umbilical vein endothelial cells.

  Anesthesia and analgesia 20120201

• Analgesia by inhibiting tetrahydrobiopterin synthesis.

  Current opinion in pharmacology 20120201

• The effects of modulating eNOS activity and coupling in ischemia/reperfusion (I/R).

  Naunyn-Schmiedeberg's archives of pharmacology 20120101

• Role of uncoupled endothelial nitric oxide synthase in abdominal aortic aneurysm formation: treatment with folic acid.

  Hypertension (Dallas, Tex. : 1979) 20120101

• Spectroscopic, catalytic and binding properties of Bacillus subtilis NO synthase-like protein: comparison with other bacterial and mammalian NO synthases.

  Journal of inorganic biochemistry 20120101

• Tetrahydrobiopterin, L-arginine and vitamin C actsynergistically to decrease oxidative stress, increase nitricoxide and improve blood flow after induction of hindlimbischemia in the rat.

  Molecular medicine (Cambridge, Mass.) 20120101

• Role of neural NO synthase (nNOS) uncoupling in the dysfunctional nitrergic vasorelaxation of penile arteries from insulin-resistant obese Zucker rats.

  PloS one 20120101

• Mechanism of cellular oxidation stress induced by asymmetric dimethylarginine.

  International journal of molecular sciences 20120101

• Effects of tetrahydrobiopterin on cerebral infarction after transient focal ischemia in rats.

  Neurological research 20111201

• Differential effects of eNOS uncoupling on conduit and small arteries in GTP-cyclohydrolase I-deficient hph-1 mice.

  American journal of physiology. Heart and circulatory physiology 20111201

• Using change in plasma phenylalanine concentrations and ability to liberalize diet to classify responsiveness to tetrahydrobiopterin therapy in patients with phenylketonuria.

  Molecular genetics and metabolism 20111201

• The tetrahydrobiopterin pathway: a novel target for the treatment of depression.

  Pharmacogenomics 20111201

• Asymmetric uptake of sepiapterin and 7,8-dihydrobiopterin as a gateway of the salvage pathway of tetrahydrobiopterin biosynthesis from the lumenal surface of rat endothelial cells.

  Molecular genetics and metabolism 20111101

• Autophagy induction by tetrahydrobiopterin deficiency.

  Autophagy 20111101

• Natural phenylalanine hydroxylase variants that confer a mild phenotype affect the enzyme's conformational stability and oligomerization equilibrium.

  Biochimica et biophysica acta 20111101

• Dual function of nitric oxide in carcinogenesis, reappraisal.

  Current drug metabolism 20111101

• Biochemical characterization of the tetrahydrobiopterin synthesis pathway in the oleaginous fungus Mortierella alpina.

  Microbiology (Reading, England) 20111101

• Disorders of BH4 metabolism and the treatment of patients with 6-pyruvoyl-tetrahydropterin synthase deficiency in Taiwan.

  Brain & development 20111101

• Sepiapterin enhances angiogenesis and functional recovery in mice after myocardial infarction.

  American journal of physiology. Heart and circulatory physiology 20111101

• Decreased tetrahydrobiopterin and disrupted association of Hsp90 with eNOS by hyperglycemia impair myocardial ischemic preconditioning.

  American journal of physiology. Heart and circulatory physiology 20111101

• [Lipid peroxidation and biomechanical properties of artery in hyperlipemia rats after treating with tetrahydrobiopterin].

  Zhongguo ying yong sheng li xue za zhi = Zhongguo yingyong shenglixue zazhi = Chinese journal of applied physiology 20111101

• Induction of vascular GTP-cyclohydrolase I and endogenous tetrahydrobiopterin synthesis protect against inflammation-induced endothelial dysfunction in human atherosclerosis.

  Circulation 20111025

• Tetrahydrobiopterin is functionally distinguishable from tetrahydrodictyopterin in Dictyostelium discoideum Ax2.

  FEBS letters 20111003

• Targeting endothelial and myocardial dysfunction with tetrahydrobiopterin.

  Journal of molecular and cellular cardiology 20111001

• GCH1, BH4 and pain.

  Current pharmaceutical biotechnology 20111001

• Bi-modal dose-dependent cardiac response to tetrahydrobiopterin in pressure-overload induced hypertrophy and heart failure.

  Journal of molecular and cellular cardiology 20111001

• Modeling of biopterin-dependent pathways of eNOS for nitric oxide and superoxide production.

  Free radical biology & medicine 20111001

• Ascorbic acid and N-acetyl cysteine prevent uncoupling of nitric oxide synthase and increase tolerance to ischemia/reperfusion injury in diabetic rat heart.

  Free radical research 20111001

• Acute localized administration of tetrahydrobiopterin and chronic systemic atorvastatin treatment restore cutaneous microvascular function in hypercholesterolaemic humans.

  The Journal of physiology 20111001

• Tetrahydrobiopterin: biochemistry and pathophysiology.

  The Biochemical journal 20110915

• Tetrahydrobiopterin improves cardiac and pulmonary function after cardiopulmonary bypass.

  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 20110901

• Gender bias in gastroparesis: is nitric oxide the answer?

  Digestive diseases and sciences 20110901

• Increased sensitivity to apoptosis induced by methotrexate is mediated by JNK.

  Arthritis and rheumatism 20110901

• Increasing dihydrobiopterin causes dysfunction of endothelial nitric oxide synthase in rats in vivo.

  American journal of physiology. Heart and circulatory physiology 20110901

• Sepiapterin improves angiogenesis of pulmonary artery endothelial cells with in utero pulmonary hypertension by recoupling endothelial nitric oxide synthase.

  American journal of physiology. Lung cellular and molecular physiology 20110901

• eNOS activation and NO function: structural motifs responsible for the posttranslational control of endothelial nitric oxide synthase activity.

  The Journal of endocrinology 20110901

• A novel high-throughput screening assay for discovery of molecules that increase cellular tetrahydrobiopterin.

  Journal of biomolecular screening 20110901

• Suppression of eNOS-derived superoxide by caveolin-1: a biopterin-dependent mechanism.

  American journal of physiology. Heart and circulatory physiology 20110901

• Synthesis and recycling of tetrahydrobiopterin in endothelial function and vascular disease.

  Nitric oxide : biology and chemistry 20110801

• Inhibition of nitric oxide synthase uncoupling by sepiapterin improves left ventricular function in streptozotocin-induced diabetic mice.

  Clinical and experimental pharmacology & physiology 20110801

• Effects of sepiapterin infusion on renal oxygenation and early acute renal injury after suprarenal aortic clamping in rats.

  Journal of cardiovascular pharmacology 20110801

• Blood phenylalanine clearance and BH(4)-responsiveness in classic phenylketonuria.

  Molecular genetics and metabolism 20110801

• Loss of NRF2 impairs gastric nitrergic stimulation and function.

  Free radical biology & medicine 20110801

• Safety of extended treatment with sapropterin dihydrochloride in patients with phenylketonuria: results of a phase 3b study.

  Molecular genetics and metabolism 20110801

• Activation of peroxisome proliferator-activated receptor-{delta} enhances regenerative capacity of human endothelial progenitor cells by stimulating biosynthesis of tetrahydrobiopterin.

  Hypertension (Dallas, Tex. : 1979) 20110801

• Tetrahydrobiopterin: important endothelial mediator independent of endothelial nitric oxide synthase.

  Hypertension (Dallas, Tex. : 1979) 20110801

• Rapid, direct effects of statin treatment on arterial redox state and nitric oxide bioavailability in human atherosclerosis via tetrahydrobiopterin-mediated endothelial nitric oxide synthase coupling.

  Circulation 20110719

• Cardiac-specific overexpression of GTP cyclohydrolase 1 restores ischaemic preconditioning during hyperglycaemia.

  Cardiovascular research 20110715

• Tetrahydrobiopterin depletion and NOS2 uncoupling contribute to heart failure-induced alterations in atrial electrophysiology.

  Cardiovascular research 20110701

• Adjusting diet with sapropterin in phenylketonuria: what factors should be considered?

  The British journal of nutrition 20110701

• Tetrahydrobiopterin deficiency and nitric oxide synthase uncoupling contribute to atherosclerosis induced by disturbed flow.

  Arteriosclerosis, thrombosis, and vascular biology 20110701

• The interplay between genotype, metabolic state and cofactor treatment governs phenylalanine hydroxylase function and drug response.

  Human molecular genetics 20110701

• GTP-cyclohydrolase deficiency responsive to sapropterin and 5-HTP supplementation: relief of treatment-refractory depression and suicidal behaviour.

  BMJ case reports 20110609

• A polymorphism of the GTP-cyclohydrolase I feedback regulator gene alters transcriptional activity and may affect response to SSRI antidepressants.

  The pharmacogenomics journal 20110601

• Nitrite supplementation reverses vascular endothelial dysfunction and large elastic artery stiffness with aging.

  Aging cell 20110601

• Dihydrofolate reductase protects endothelial nitric oxide synthase from uncoupling in tetrahydrobiopterin deficiency.

  Free radical biology & medicine 20110601

• Elevated oxidative stress and endothelial dysfunction in right coronary artery of right ventricular hypertrophy.

  Journal of applied physiology (Bethesda, Md. : 1985) 20110601

• Diagnosis of tetrahydrobiopterin deficiency using filter paper blood spots: further development of the method and 5 years experience.

  Journal of inherited metabolic disease 20110601

• Tetrahydrobiopterin restores diastolic function and attenuates superoxide production in ovariectomized mRen2.Lewis rats.

  Endocrinology 20110601

• Role of tetrahydrobiopterin (BH4) in hyperhomocysteinemia-induced endothelial dysfuction: new indication for this orphan-drug?

  American journal of physiology. Endocrinology and metabolism 20110601

• A yeast-based screen reveals that sulfasalazine inhibits tetrahydrobiopterin biosynthesis.

  Nature chemical biology 20110601

• Tetrahydrobiopterin attenuates superoxide-induced reduction in nitric oxide.

  Frontiers in bioscience (Scholar edition) 20110601

• [Phenylketonuria (PKU) in Iceland].

  Laeknabladid 20110601

• Tetrahydrobiopterin and phenylketonuria.

  The Journal of pediatrics 20110501

• Tetrahydrobiopterin responsiveness of patients with phenylalanine hydroxylase deficiency.

  Jornal de pediatria 20110501

• Role of increased guanosine triphosphate cyclohydrolase-1 expression and tetrahydrobiopterin levels upon T cell activation.

  The Journal of biological chemistry 20110422

• Pathogenetic role of eNOS uncoupling in cardiopulmonary disorders.

  Free radical biology & medicine 20110401

• Tetrahydrobiopterin is present in high quantity in human milk and has a vasorelaxing effect on newborn rat mesenteric arteries.

  Pediatric research 20110401

• The effect of blood phenylalanine concentration on Kuvan™ response in phenylketonuria.

  Molecular genetics and metabolism 20110401

• Pyridoxal 5'-phosphate in cerebrospinal fluid; factors affecting concentration.

  Journal of inherited metabolic disease 20110401

• Molecular characterization of phenylketonuria and tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency in Japan.

  Journal of human genetics 20110401

• Formation of the iron-oxo hydroxylating species in the catalytic cycle of aromatic amino acid hydroxylases.

  Chemistry (Weinheim an der Bergstrasse, Germany) 20110321

• Tetrahydrobiopterin therapy for phenylketonuria in infants and young children.

  The Journal of pediatrics 20110301

• Reduction of radiation-induced vascular nitrosative stress by the vitamin E analog γ-tocotrienol: evidence of a role for tetrahydrobiopterin.

  International journal of radiation oncology, biology, physics 20110301

• Tetrahydrobiopterin and PKU: into the future.

  The Journal of pediatrics 20110301

• Antihypertensive therapy increases tetrahydrobiopterin levels and NO/cGMP signaling in small arteries of angiotensin II-infused hypertensive rats.

  American journal of physiology. Heart and circulatory physiology 20110301

• Reversal of inducible nitric oxide synthase uncoupling unmasks tolerance to ischemia/reperfusion injury in the diabetic rat heart.

  Journal of molecular and cellular cardiology 20110301

• Tetrahydrobiopterin and hypertension: more than an emigrating story.

  American journal of physiology. Heart and circulatory physiology 20110301

• Identification and characterization of an inborn error of metabolism caused by dihydrofolate reductase deficiency.

  American journal of human genetics 20110211

• Methemoglobinemia caused by 8-aminoquinoline drugs: DFT calculations suggest an analogy to H4B's role in nitric oxide synthase.

  Journal of the American Chemical Society 20110209

• Phenotyping and treatment of phenylketonuria.

  Lancet (London, England) 20110205

• Safety of sapropterin dihydrochloride (6r-bh4) in patients with pulmonary hypertension.

  Experimental lung research 20110201

• Molecular genetics and impact of residual in vitro phenylalanine hydroxylase activity on tetrahydrobiopterin responsiveness in Turkish PKU population.

  Molecular genetics and metabolism 20110201

• Tetrahydrobiopterin analogues with NO-dependent pulmonary vasodilator properties.

  European journal of pharmacology 20110110

• Endothelin-1 antagonism and nitric oxide augmentation prevents cyclosporine-induced vasomotor impairment.

  The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 20110101

• Activation of NAD(P)H oxidases by thromboxane A2 receptor uncouples endothelial nitric oxide synthase.

  Arteriosclerosis, thrombosis, and vascular biology 20110101

• Membrane transport of sepiapterin and dihydrobiopterin by equilibrative nucleoside transporters: a plausible gateway for the salvage pathway of tetrahydrobiopterin biosynthesis.

  Molecular genetics and metabolism 20110101

• Cilostazol inhibits cytokine-induced tetrahydrobiopterin biosynthesis in human umbilical vein endothelial cells.

  Journal of atherosclerosis and thrombosis 20110101

• Effect of tetrahydrobiopterin on Phe/Tyr ratios and variation in Phe levels in tetrahydrobiopterin responsive PKU patients.

  Molecular genetics and metabolism 20110101

• Phenylalanine hydroxylase deficiency: molecular epidemiology and predictable BH4-responsiveness in South Portugal PKU patients.

  Molecular genetics and metabolism 20110101

• Diagnosis, classification, and genetics of phenylketonuria and tetrahydrobiopterin (BH4) deficiencies.

  Molecular genetics and metabolism 20110101

• Up to date knowledge on different treatment strategies for phenylketonuria.

  Molecular genetics and metabolism 20110101

• The 48-hour tetrahydrobiopterin loading test in patients with phenylketonuria: evaluation of protocol and influence of baseline phenylalanine concentration.

  Molecular genetics and metabolism 20110101

• Optimized loading test to evaluate responsiveness to tetrahydrobiopterin (BH4) in Brazilian patients with phenylalanine hydroxylase deficiency.

  Molecular genetics and metabolism 20110101

• Searching for the protein targets of bioactive molecules.

  Chimia 20110101

• New role for L-arginine in regulation of inducible nitric-oxide-synthase-derived superoxide anion production in raw 264.7 macrophages.

  TheScientificWorldJournal 20110101

• [Nitric oxide and preeclampsia].

  Orvosi hetilap 20101226

• Tetrahydrobiopterin: pleiotropic roles in cardiovascular pathophysiology.

  Heart (British Cardiac Society) 20101201

• Homocysteine impairs coronary artery endothelial function by inhibiting tetrahydrobiopterin in patients with hyperhomocysteinemia.

  American journal of physiology. Endocrinology and metabolism 20101201

• Tetrahydrobiopterin does not affect end-organ responsiveness to norepinephrine-mediated vasoconstriction in aged skin.

  American journal of physiology. Regulatory, integrative and comparative physiology 20101201

• Endothelial, sympathetic, and cardiac function in inherited (6R)-L-erythro-5,6,7,8-tetrahydro-L-biopterin deficiency.

  Circulation. Cardiovascular genetics 20101201

• BH(4) therapy impacts the nutrition status and intake in children with phenylketonuria: 2-year follow-up.

  Journal of inherited metabolic disease 20101201

• GTP cyclohydrolase I/BH4 pathway protects EPCs via suppressing oxidative stress and thrombospondin-1 in salt-sensitive hypertension.

  Hypertension (Dallas, Tex. : 1979) 20101201

• Advances and challenges in phenylketonuria.

  Journal of inherited metabolic disease 20101201

• Letter by Antoniades et al regarding article, 'Uncoupled cardiac nitric oxide synthase mediates diastolic dysfunction'.

  Circulation 20101123

• New insights into tetrahydrobiopterin pharmacodynamics from Pah enu1/2, a mouse model for compound heterozygous tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency.

  Biochemical pharmacology 20101115

• Novel strategies to ameliorate radiation injury: a possible role for tetrahydrobiopterin.

  Current drug targets 20101101

• Dihydropteridine reductase activity in the brainstem of intrauterine growth-restricted rats.

  International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience 20101101

• Roles of tetrahydrobiopterin in promoting tumor angiogenesis.

  The American journal of pathology 20101101

• Lessons from 30 years of selective screening for tetrahydrobiopterin deficiency.

  Journal of inherited metabolic disease 20101001

• Resveratrol reverses endothelial nitric-oxide synthase uncoupling in apolipoprotein E knockout mice.

  The Journal of pharmacology and experimental therapeutics 20101001

• Activation of phenylalanine hydroxylase induces positive cooperativity toward the natural cofactor.

  The Journal of biological chemistry 20101001

• Donor pretreatment with tetrahydrobiopterin saves pancreatic isografts from ischemia reperfusion injury in a mouse model.

  American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 20101001

• Hepatic endothelial dysfunction and abnormal angiogenesis: new targets in the treatment of portal hypertension.

  Journal of hepatology 20100901

• Nitric oxide pathway in hypertrophied heart: new therapeutic uses of nitric oxide donors.

  Journal of hypertension 20100901

• Maintenance of cellular tetrahydrobiopterin homeostasis.

  BMB reports 20100901

• Alterations in dopamine and glutamate neurotransmission in tetrahydrobiopterin deficient spr-/- mice: relevance to schizophrenia.

  BMB reports 20100901

• Nitric oxide synthase stabilizes the tetrahydrobiopterin cofactor radical by controlling its protonation state.

  Journal of the American Chemical Society 20100825

• Mechanism of dihydrofolate reductase downregulation in melanoma by 3-O-(3,4,5-trimethoxybenzoyl)-(-)-epicatechin.

  Journal of cellular biochemistry 20100815

• Tetrahydrobiopterin and the regulation of hypoxic pulmonary vasoconstriction.

  The European respiratory journal 20100801

• Nitric oxide synthase 3 contributes to ventilator-induced lung injury.

  American journal of physiology. Lung cellular and molecular physiology 20100801

• Oral tetrahydrobiopterin improves the beneficial effect of adenoviral-mediated eNOS gene transfer after induction of hindlimb ischemia.

  Molecular therapy : the journal of the American Society of Gene Therapy 20100801

• Challenges and pitfalls in the management of phenylketonuria.

  Pediatrics 20100801

• Sapropterin dihydrochloride: a new drug and a new concept in the management of phenylketonuria.

  Drugs of today (Barcelona, Spain : 1998) 20100801

• Endothelial dysfunction: potential clinical implications.

  Minerva medica 20100801

• Introduction of sapropterin dihydrochloride as standard of care in patients with phenylketonuria.

  Molecular genetics and metabolism 20100701

• Cardiac myocyte-specific overexpression of human GTP cyclohydrolase I protects against acute cardiac allograft rejection.

  American journal of physiology. Heart and circulatory physiology 20100701

• Tetrahydrobiopterin as a novel therapeutic intervention for autism.

  Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics 20100701

• Potential therapeutic applications of tetrahydrobiopterin: from inherited hyperphenylalaninemia to mitochondrial diseases.

  Annals of the New York Academy of Sciences 20100701

• Sapropterin dihydrochloride for phenylketonuria.

  The Cochrane database of systematic reviews 20100616

• Synthesis of poly(methyl methacrylate) core/chitosan-mixed-polyethyleneimine shell nanoparticles and their antibacterial property.

  Colloids and surfaces. B, Biointerfaces 20100601

• Sapropterin.

  British journal of clinical pharmacology 20100601

• [The Dental and Pharmaceutical Benefits Agency misinterpret its mission].

  Lakartidningen 20100601

• Sapropterin. Phenylketonuria: for a minority of patients.

  Prescrire international 20100601

• Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo.

  Human molecular genetics 20100515

• Tetrahydrobiopterin supplementation reduces atherosclerosis and vascular inflammation in apolipoprotein E-knockout mice.

  Clinical science (London, England : 1979) 20100506

• The role of nitric oxide in the expression of renal aquaporin 2 in a cirrhotic rat model: does an AVP-independent mechanism exist for the regulation of AQP2 expression?

  Digestive diseases and sciences 20100501

• Unresponsiveness to tetrahydrobiopterin of phenylalanine hydroxylase deficiency.

  Metabolism: clinical and experimental 20100501

• Molecular mechanisms underlying the activation of eNOS.

  Pflugers Archiv : European journal of physiology 20100501

• L-arginine and antioxidant diet supplementation partially restores nitric oxide-dependent regulation of phenylephrine renal vasoconstriction in diabetics rats.

  Journal of renal nutrition : the official journal of the Council on Renal Nutrition of the National Kidney Foundation 20100501

• An important role of increase in tetrahydrobiopterin via H2O2-JAK2 signalling pathway in late phase of ischaemic preconditioning.

  Experimental physiology 20100501

• Tetrahydrobiopterin (BH4), a cofactor for nNOS, restores gastric emptying and nNOS expression in female diabetic rats.

  American journal of physiology. Gastrointestinal and liver physiology 20100501

• Endothelial dysfunction in diabetes: multiple targets for treatment.

  Pflugers Archiv : European journal of physiology 20100501

• Identification and optimization of tyrosine hydroxylase activity in Mucuna pruriens DC. var. utilis.

  Planta 20100501

• Nitric oxide and oxidative stress in vascular disease.

  Pflugers Archiv : European journal of physiology 20100501

• Caloric restriction reverses high-fat diet-induced endothelial dysfunction and vascular superoxide production in C57Bl/6 mice.

  Heart and vessels 20100501

• Localized tyrosine or tetrahydrobiopterin supplementation corrects the age-related decline in cutaneous vasoconstriction.

  The Journal of physiology 20100415

• Peroxynitrite induces destruction of the tetrahydrobiopterin and heme in endothelial nitric oxide synthase: transition from reversible to irreversible enzyme inhibition.

  Biochemistry 20100413

• Improvement of impaired endothelial function by tetrahydrobiopterin in stroke-prone spontaneously hypertensive rats.

  European journal of pharmacology 20100410

• Alterations in the modulation of cerebrovascular tone and blood flow by nitric oxide synthases in SHRsp with stroke.

  Cardiovascular research 20100401

• Long-chain polyunsaturated fatty acid status in phenylketonuric patients treated with tetrahydrobiopterin.

  Clinical biochemistry 20100301

• Hyperglycemia adversely modulates endothelial nitric oxide synthase during anesthetic preconditioning through tetrahydrobiopterin- and heat shock protein 90-mediated mechanisms.

  Anesthesiology 20100301

• Mass spectroscopy and molecular modeling predict endothelial nitric oxide synthase dimer collapse by hydrogen peroxide through zinc tetrathiolate metal-binding site disruption.

  DNA and cell biology 20100301

• Reactive oxygen and nitrogen species regulate inducible nitric oxide synthase function shifting the balance of nitric oxide and superoxide production.

  Archives of biochemistry and biophysics 20100215

• GTP cyclohydrolase I phosphorylation and interaction with GTP cyclohydrolase feedback regulatory protein provide novel regulation of endothelial tetrahydrobiopterin and nitric oxide.

  Circulation research 20100205

• Uncoupled cardiac nitric oxide synthase mediates diastolic dysfunction.

  Circulation 20100202

• NO way to relax: the complexities of coupling nitric oxide synthase pathways in the heart.

  Circulation 20100202

• An enzymatic method to distinguish tetrahydrobiopterin from oxidized biopterins using UDP-glucose:tetrahydrobiopterin glucosyltransferase.

  Analytical biochemistry 20100201

• Telmisartan improves endothelial dysfunction and renal autoregulation in Dahl salt-sensitive rats.

  Hypertension research : official journal of the Japanese Society of Hypertension 20100201

• A weapon of endothelial cells for fighting vascular disease.

  Hypertension research : official journal of the Japanese Society of Hypertension 20100201

• Tetrahydrobiopterin responsiveness after extended loading test of 12 Danish PKU patients with the Y414C mutation.

  Journal of inherited metabolic disease 20100201

• Decreased arteriolar tetrahydrobiopterin is linked to superoxide generation from nitric oxide synthase in mice fed high salt.

  Microcirculation (New York, N.Y. : 1994) 20100201

• Relative bioavailability of sapropterin from intact and dissolved sapropterin dihydrochloride tablets and the effects of food: a randomized, open-label, crossover study in healthy adults.

  Clinical therapeutics 20100201

• Arginase contributes to endothelial cell oxidative stress in response to plasma from women with preeclampsia.

  Cardiovascular research 20100101

• The effects of tetrahydrobiopterin on intracerebral hemorrhage-induced brain injury in mice.

  Acta neurochirurgica. Supplement 20100101

• Regulation of nitric oxide production in health and disease.

  Current opinion in clinical nutrition and metabolic care 20100101

• Evidence against tetrahydrobiopterin depletion of vascular tissue exposed to nitric oxide/superoxide or nitroglycerin.

  Free radical biology & medicine 20100101

• Thiol-metabolizing proteins and endothelial redox state: differential modulation of eNOS and biopterin pathways.

  American journal of physiology. Heart and circulatory physiology 20100101

• The tetrahydrobiopterin pathway and pain.

  Current opinion in investigational drugs (London, England : 2000) 20100101

• [Phenylketonuria--toward a better carry-over care].

  Nihon rinsho. Japanese journal of clinical medicine 20100101

• Cognitive functions in patients with phenylketonuria in long-term treatment with tetrahydrobiopterin.

  Molecular genetics and metabolism 20100101

• Clear correlation of tetrahydrobiopterin with nitric oxide bioavailability in continuous ambulatory peritoneal dialysis.

  Blood purification 20100101

• Reaching out to the lost generation of adults with early-treated phenylketonuria (PKU).

  Molecular genetics and metabolism 20100101

• Sapropterin therapy increases stability of blood phenylalanine levels in patients with BH4-responsive phenylketonuria (PKU).

  Molecular genetics and metabolism 20100101

• Regulation of endothelial cell tetrahydrobiopterin pathophysiological and therapeutic implications.

  Advances in pharmacology (San Diego, Calif.) 20100101

• Impaired endothelial function may be due to decreased aortic tetrahydrobiopterin, assessed by a new flow-mediated vasodilation in vivo in hypercholesterolemic/atherogenic mice.

  Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 20091201

• Identification and functional characterization of phosphorylation sites on GTP cyclohydrolase I.

  Arteriosclerosis, thrombosis, and vascular biology 20091201

• Active NF-E2-related factor (Nrf2) contributes to keep endothelial NO synthase (eNOS) in the coupled state: role of reactive oxygen species (ROS), eNOS, and heme oxygenase (HO-1) levels.

  The Journal of biological chemistry 20091113

• Ascorbic acid and tetrahydrobiopterin potentiate the EDHF phenomenon by generating hydrogen peroxide.

  Cardiovascular research 20091101

• Examination of tetrahydrobiopterin pathway genes in autism.

  Genes, brain, and behavior 20091101

• Role of tetrahydrobiopterin in resistance to myocardial ischemia in Brown Norway and Dahl S rats.

  American journal of physiology. Heart and circulatory physiology 20091101

• Diminished expression of dihydropteridine reductase is a potent biomarker for hypertensive vessels.

  Proteomics 20091101

• Ascorbic acid and tetrahydrobiopterin: looking beyond nitric oxide bioavailability.

  Cardiovascular research 20091101

• Adverse ventricular remodeling and exacerbated NOS uncoupling from pressure-overload in mice lacking the beta3-adrenoreceptor.

  Journal of molecular and cellular cardiology 20091101

• eNOS uncoupling and endothelial dysfunction in aged vessels.

  American journal of physiology. Heart and circulatory physiology 20091101

• Tetrahydrobiopterin, superoxide, and vascular dysfunction.

  Free radical biology & medicine 20091015

• Critical role for tetrahydrobiopterin recycling by dihydrofolate reductase in regulation of endothelial nitric-oxide synthase coupling: relative importance of the de novo biopterin synthesis versus salvage pathways.

  The Journal of biological chemistry 20091009

• Do sauna therapy and exercise act by raising the availability of tetrahydrobiopterin?

  Medical hypotheses 20091001

• Optimization of continuous in vivo DOPA production and studies on ectopic DA synthesis using rAAV5 vectors in Parkinsonian rats.

  Journal of neurochemistry 20091001

• Nitric oxide and pain: 'Something old, something new'.

  Acta anaesthesiologica Scandinavica 20091001

• [Fusion of field and laboratory studies on the investigation of arsenic].

  Yakugaku zasshi : Journal of the Pharmaceutical Society of Japan 20091001

• Inhibition of CDKS by roscovitine suppressed LPS-induced *NO production through inhibiting NFkappaB activation and BH4 biosynthesis in macrophages.

  American journal of physiology. Cell physiology 20090901

• Tyrosine nitration of PA700 activates the 26S proteasome to induce endothelial dysfunction in mice with angiotensin II-induced hypertension.

  Hypertension (Dallas, Tex. : 1979) 20090901

• Sapropterin: a new therapeutic agent for phenylketonuria.

  The Annals of pharmacotherapy 20090901

• [Research advance in the treatment of phenylketonuria].

  Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics 20090901

• Tetrahydrobiopterin in the prevention of hypertonia in hypoxic fetal brain.

  Annals of neurology 20090901

• Determination of marker pteridins and biopterin reduced forms, tetrahydrobiopterin and dihydrobiopterin, in human urine, using a post-column photoinduced fluorescence liquid chromatographic derivatization method.

  Analytica chimica acta 20090819

• High-cholesterol diet augments endothelial dysfunction via elevated oxidative stress and reduced tetrahydrobiopterin in Ins2(Akita) mice, an autosomal dominant mutant type 1 diabetic model.

  Clinical and experimental pharmacology & physiology 20090801

• GTP cyclohydrolase I expression is regulated by nitric oxide: role of cyclic AMP.

  American journal of physiology. Lung cellular and molecular physiology 20090801

• Local tetrahydrobiopterin administration augments cutaneous vasoconstriction in aged humans.

  The Journal of physiology 20090801

• Blood phenylalanine concentrations in patients with PAH-deficient hyperphenylalaninaemia off diet without and with three different single oral doses of tetrahydrobiopterin: assessing responsiveness in a model of statistical process control.

  Journal of inherited metabolic disease 20090801

• Effects of ageing and exercise training on eNOS uncoupling in skeletal muscle resistance arterioles.

  The Journal of physiology 20090801

• Exercising an option to prevent age related decline of vascular BH4 and uncoupling of eNOS.

  The Journal of physiology 20090801

• Detection of tetrahydrobiopterin by LC-MS/MS in plasma from multiple species.

  Bioanalysis 20090801

• [Biomechanical properties of artery in spontaneously hypertensive rats after treating with tetrahydrobiopterin].

  Zhongguo ying yong sheng li xue za zhi = Zhongguo yingyong shenglixue zazhi = Chinese journal of applied physiology 20090801

• Expression analysis of the aldo-keto reductases involved in the novel biosynthetic pathway of tetrahydrobiopterin in human and mouse tissues.

  Journal of biochemistry 20090701

• Genotype-predicted tetrahydrobiopterin (BH4)-responsiveness and molecular genetics in Croatian patients with phenylalanine hydroxylase (PAH) deficiency.

  Molecular genetics and metabolism 20090701

• Sepiapterin reductase regulation of endothelial tetrahydrobiopterin and nitric oxide bioavailability.

  American journal of physiology. Heart and circulatory physiology 20090701

• Exhaustive analysis of BH4 and dopamine biosynthesis genes in patients with Dopa-responsive dystonia.

  Brain : a journal of neurology 20090701

• Disorders of biopterin metabolism.

  Journal of inherited metabolic disease 20090601

• Sepiapterin decreases acute rejection and apoptosis in cardiac transplants independently of changes in nitric oxide and inducible nitric-oxide synthase dimerization.

  The Journal of pharmacology and experimental therapeutics 20090601

• Endothelium-specific GTP cyclohydrolase I overexpression accelerates refractory wound healing by suppressing oxidative stress in diabetes.

  American journal of physiology. Endocrinology and metabolism 20090601

• PTR1-dependent synthesis of tetrahydrobiopterin contributes to oxidant susceptibility in the trypanosomatid protozoan parasite Leishmania major.

  Current genetics 20090601

• Tetrahydrobiopterin, endothelial nitric oxide synthase, and mitochondrial function in the heart.

  Hypertension (Dallas, Tex. : 1979) 20090601

• Metallothionein abrogates GTP cyclohydrolase I inhibition-induced cardiac contractile and morphological defects: role of mitochondrial biogenesis.

  Hypertension (Dallas, Tex. : 1979) 20090601

• A limited spectrum of phenylalanine hydroxylase mutations is observed in phenylketonuria patients in western Poland and implications for treatment with 6R tetrahydrobiopterin.

  Journal of human genetics 20090601

• Novel therapeutic strategies targeting vascular redox in human atherosclerosis.

  Recent patents on cardiovascular drug discovery 20090601

• GTP cyclohydrolase I expression, protein, and activity determine intracellular tetrahydrobiopterin levels, independent of GTP cyclohydrolase feedback regulatory protein expression.

  The Journal of biological chemistry 20090515

• Tetrahydrobiopterin recycling, a key determinant of endothelial nitric-oxide synthase-dependent signaling pathways in cultured vascular endothelial cells.

  The Journal of biological chemistry 20090508

• L-arginine and tetrahydrobiopterin protects against ischemia/reperfusion-induced endothelial dysfunction in patients with type 2 diabetes mellitus and coronary artery disease.

  Atherosclerosis 20090501

• Dietary nitrite prevents hypercholesterolemic microvascular inflammation and reverses endothelial dysfunction.

  American journal of physiology. Heart and circulatory physiology 20090501

• Efficacy of sapropterin dihydrochloride in increasing phenylalanine tolerance in children with phenylketonuria: a phase III, randomized, double-blind, placebo-controlled study.

  The Journal of pediatrics 20090501

• Flow-induced dilation is mediated by Akt-dependent activation of endothelial nitric oxide synthase-derived hydrogen peroxide in mouse cerebral arteries.

  Stroke 20090501

• Detrimental effect of combined exercise training and eNOS overexpression on cardiac function after myocardial infarction.

  American journal of physiology. Heart and circulatory physiology 20090501

• Plasma tetrahydrobiopterin/dihydrobiopterin ratio: a possible marker of endothelial dysfunction.

  Circulation journal : official journal of the Japanese Circulation Society 20090501

• Effects of tetrahydrobiopterin on venous bubble grade and acute diving-induced changes in cardiovascular function.

  Clinical physiology and functional imaging 20090401

• Effect of uncoupling endothelial nitric oxide synthase on calcium homeostasis in aged porcine endothelial cells.

  Cardiovascular research 20090401

• Optimizing the use of sapropterin (BH(4)) in the management of phenylketonuria.

  Molecular genetics and metabolism 20090401

• Proteomic analysis of GTP cyclohydrolase 1 multiprotein complexes in cultured normal adult human astrocytes under both basal and cytokine-activated conditions.

  Proteomics 20090401

• Physarum nitric oxide synthases: genomic structures and enzymology of recombinant proteins.

  The Biochemical journal 20090315

• Comparative computational analysis of active and inactive cofactors of nitric oxide synthase.

  The journal of physical chemistry. B 20090312

• The solution electrochemistry of tetrahydrobiopterin revisited.

  Nitric oxide : biology and chemistry 20090301

• Protective effect of bromocriptine against BH4-induced Cath.a cell death involving up-regulation of antioxidant enzymes.

  Neuroscience letters 20090227

• Biochemical characterization of mutant phenylalanine hydroxylase enzymes and correlation with clinical presentation in hyperphenylalaninaemic patients.

  Journal of inherited metabolic disease 20090201

• Tetrahydrobiopterin deficiency in human rabies.

  Journal of inherited metabolic disease 20090201

• Significance of genotype in tetrahydrobiopterin-responsive phenylketonuria.

  Journal of inherited metabolic disease 20090201

• Transcellular relocation of tetrahydrobiopterin across Caco-2 cells: a model study of tetrahydrobiopterin absorption through epithelial cells of intestinal mucosa.

  Journal of inherited metabolic disease 20090201

• Pharmacokinetics of tetrahydrobiopterin following oral loadings with three single dosages in patients with phenylketonuria.

  Journal of inherited metabolic disease 20090201

• Tetrahydrobiopterin in intestinal lumen: its absorption and secretion in the small intestine and the elimination in the large intestine.

  Journal of inherited metabolic disease 20090201

• Effect of BH(4) supplementation on phenylalanine tolerance.

  Journal of inherited metabolic disease 20090201

• H(2)O(2) increases de novo synthesis of (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin via GTP cyclohydrolase I and its feedback regulatory protein in vitiligo.

  Journal of inherited metabolic disease 20090201

• A new role for caveolin-1: regulation of guanosine triphosphate cyclohydrolase I and tetrahydrobiopterin in endothelial cells.

  Hypertension (Dallas, Tex. : 1979) 20090201

• Guanosine triphosphate cyclohydrolase I expression and enzymatic activity are present in caveolae of endothelial cells.

  Hypertension (Dallas, Tex. : 1979) 20090201

• Tetrahydrobiopterin in biomedical research.

  Journal of inherited metabolic disease 20090201

• Proceedings of the International Conference on Tetrahydrobiopterin, Phenylketonuria, and Nitric Oxide Synthase, St. Moritz/Champér, Switzerland, March 23-28, 2008.

  Journal of inherited metabolic disease 20090201

• Reactive oxygen species production and MAPK activation are implicated in tetrahydrobiopterin-induced SH-SY5Y cell death.

  Neuroscience letters 20090116

• Quantitative regulation of intracellular endothelial nitric-oxide synthase (eNOS) coupling by both tetrahydrobiopterin-eNOS stoichiometry and biopterin redox status: insights from cells with tet-regulated GTP cyclohydrolase I expression.

  The Journal of biological chemistry 20090109

• Glyceryl ether monooxygenase resembles aromatic amino acid hydroxylases in metal ion and tetrahydrobiopterin dependence.

  Biological chemistry 20090101

• Vascular protection by tetrahydrobiopterin: progress and therapeutic prospects.

  Trends in pharmacological sciences 20090101

• [Biopterin and child neurologic disease].

  No to hattatsu = Brain and development 20090101

• [Study of the photochemical properties of biopterin and its reduced forms].

  Prikladnaia biokhimiia i mikrobiologiia 20090101

• Nitric oxide and vascular insulin resistance.

  BioFactors (Oxford, England) 20090101

• Sapropterin: a review of its use in the treatment of primary hyperphenylalaninaemia.

  Drugs 20090101

• [Genotyping and treatment modification in patients with phenylketonuria: an introduction to pharmacogenomics].

  Przeglad lekarski 20090101

• [A novel strategy to design specific iNOS inhibitors].

  Medecine sciences : M/S 20090101

• Spotlight on sapropterin in primary hyperphenylalaninemia.

  BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapy 20090101

• Cross-sectional assessment of the consequences of a GTP cyclohydrolase 1 haplotype for specialized tertiary outpatient pain care.

  The Clinical journal of pain 20090101

• Changing dietary practices in phenylketonuria.

  The Turkish journal of pediatrics 20090101

• Angiotensin II type 1 receptor blocker ameliorates uncoupled endothelial nitric oxide synthase in rats with experimental diabetic nephropathy.

  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 20081201

• Superoxide dismutase restores eNOS expression and function in resistance pulmonary arteries from neonatal lambs with persistent pulmonary hypertension.

  American journal of physiology. Lung cellular and molecular physiology 20081201

• Bringing down the ROS: a new therapeutic approach for PPHN.

  American journal of physiology. Lung cellular and molecular physiology 20081201

• Maternal phenylketonuria and tetrahydrobiopterin.

  Pediatrics 20081201

• Diagnosis of tetrahydrobiopterin (BH4) responsive mild phenylketonuria in Japan over the past 10 years.

  Annals of the Academy of Medicine, Singapore 20081201

• Stabilization and characterization of a heme-oxy reaction intermediate in inducible nitric-oxide synthase.

  The Journal of biological chemistry 20081128

• Crystal structure of tryptophan hydroxylase with bound amino acid substrate.

  Biochemistry 20081118

• Safety and efficacy of 22 weeks of treatment with sapropterin dihydrochloride in patients with phenylketonuria.

  American journal of medical genetics. Part A 20081115

• Direct binding of GTP cyclohydrolase and tyrosine hydroxylase: regulatory interactions between key enzymes in dopamine biosynthesis.

  The Journal of biological chemistry 20081114

• Reduced hyperalgesia in homozygous carriers of a GTP cyclohydrolase 1 haplotype.

  European journal of pain (London, England) 20081101

• Dietary supplementation with cholesterol and docosahexaenoic acid increases the activity of the arginine-nitric oxide pathway in tissues of young pigs.

  Nitric oxide : biology and chemistry 20081101

• Laminar shear stress inhibits lipid peroxidation induced by high glucose plus arachidonic acid in endothelial cells.

  American journal of physiology. Heart and circulatory physiology 20081101

• Deficient BH4 production via de novo and salvage pathways regulates NO responses to cytokines in adult cardiac myocytes.

  American journal of physiology. Heart and circulatory physiology 20081101

• A polarizable force-field model for quantum-mechanical-molecular-mechanical Hamiltonian using expansion of point charges into orbitals.

  The Journal of chemical physics 20081021

• Ascorbate sustains neutrophil NOS expression, catalysis, and oxidative burst.

  Free radical biology & medicine 20081015

• Air pollution exposure potentiates hypertension through reactive oxygen species-mediated activation of Rho/ROCK.

  Arteriosclerosis, thrombosis, and vascular biology 20081001

• Cytokines and lipopolysaccharides induce inducible nitric oxide synthase but not enzyme activity in adult rat cardiomyocytes.

  Free radical biology & medicine 20081001

• Perivascular nitric oxide and superoxide in neonatal cerebral hypoxia-ischemia.

  American journal of physiology. Heart and circulatory physiology 20081001

• CCR2-mediated antiinflammatory effects of endothelial tetrahydrobiopterin inhibit vascular injury-induced accelerated atherosclerosis.

  Circulation 20080930

• Evaluation of neonatal BH4 loading test in neonates screened for hyperphenylalaninemia.

  Early human development 20080901

• Acute inhibition of guanosine triphosphate cyclohydrolase 1 uncouples endothelial nitric oxide synthase and elevates blood pressure.

  Hypertension (Dallas, Tex. : 1979) 20080901

• Renin inhibitor aliskiren improves impaired nitric oxide bioavailability and protects against atherosclerotic changes.

  Hypertension (Dallas, Tex. : 1979) 20080901

• Beneficial effects of exogenous tetrahydrobiopterin on left ventricular remodeling after myocardial infarction in rats: the possible role of oxidative stress caused by uncoupled endothelial nitric oxide synthase.

  Circulation journal : official journal of the Japanese Circulation Society 20080901

• Chronic immune stimulation correlates with reduced phenylalanine turnover.

  Current drug metabolism 20080901

• The methylation, neurotransmitter, and antioxidant connections between folate and depression.

  Alternative medicine review : a journal of clinical therapeutic 20080901

• Serum phenylalanine in patients post trauma and with sepsis correlate to neopterin concentrations.

  Amino acids 20080801

• Cellular uptake of sepiapterin and push-pull accumulation of tetrahydrobiopterin.

  Molecular genetics and metabolism 20080801

• Three-day tetrahydrobiopterin therapy increases in vivo hepatic NOS activity and reduces portal pressure in CCl4 cirrhotic rats.

  Journal of hepatology 20080801

• Loss of function in phenylketonuria is caused by impaired molecular motions and conformational instability.

  American journal of human genetics 20080711

• Regulation of eNOS-derived superoxide by endogenous methylarginines.

  Biochemistry 20080708

• GCH1 haplotype determines vascular and plasma biopterin availability in coronary artery disease effects on vascular superoxide production and endothelial function.

  Journal of the American College of Cardiology 20080708

• Tetrahydrobiopterin shows chaperone activity for tyrosine hydroxylase.

  Journal of neurochemistry 20080701

• Exposure to inhaled particulate matter impairs cardiac function in senescent mice.

  American journal of physiology. Regulatory, integrative and comparative physiology 20080701

• A plaidoyer for cutaneous enzymology: our view of some important unanswered questions on the contributions of selected key enzymes to epidermal homeostasis.

  Experimental dermatology 20080701

• Development of a model for assessment of phenylalanine hydroxylase activity in newborns with phenylketonuria receiving tetrahydrobiopterin: a potential for practical implementation.

  Molecular genetics and metabolism 20080701

• Erythropoietin increases endothelial biosynthesis of tetrahydrobiopterin by activation of protein kinase B alpha/Akt1.

  Hypertension (Dallas, Tex. : 1979) 20080701

• New drugs08, part 2.

  Nursing 20080701

• Sapropterin (Kuvan) for phenylketonuria.

  The Medical letter on drugs and therapeutics 20080602

• Nitric oxide, tetrahydrobiopterin, oxidative stress, and endothelial dysfunction in hypertension.

  Antioxidants & redox signaling 20080601

• Tetrahydrobiopterin: a novel antihypertensive therapy.

  Journal of human hypertension 20080601

• Reduced vascular tetrahydrobiopterin (BH4) and endothelial function with ageing: is it time for a chronic BH4 supplementation trial in middle-aged and older adults?

  The Journal of physiology 20080601

• Augmentation of vascular remodeling by uncoupled endothelial nitric oxide synthase in a mouse model of diabetes mellitus.

  Arteriosclerosis, thrombosis, and vascular biology 20080601

• Vascular oxidative stress and nitric oxide depletion in HIV-1 transgenic rats are reversed by glutathione restoration.

  American journal of physiology. Heart and circulatory physiology 20080601

• Effect of pioglitazone on nitroglycerin-induced impairment of nitric oxide bioavailability by a catheter-type nitric oxide sensor.

  Circulation journal : official journal of the Japanese Circulation Society 20080601

• Role of Phe-99 and Trp-196 of sepiapterin reductase from Chlorobium tepidum in the production of L-threo-tetrahydrobiopterin.

  Acta biochimica et biophysica Sinica 20080601

• [BH4 in the management of phenylketonuria].

  Archives de pediatrie : organe officiel de la Societe francaise de pediatrie 20080601

• Reversal of cardiac hypertrophy and fibrosis from pressure overload by tetrahydrobiopterin: efficacy of recoupling nitric oxide synthase as a therapeutic strategy.

  Circulation 20080520

• Mechanisms underlying recoupling of eNOS by HMG-CoA reductase inhibition in a rat model of streptozotocin-induced diabetes mellitus.

  Atherosclerosis 20080501

• Regulation of melanogenesis--controversies and new concepts.

  Experimental dermatology 20080501

• The adverse cardiopulmonary phenotype of caveolin-1 deficient mice is mediated by a dysfunctional endothelium.

  Journal of molecular and cellular cardiology 20080501

• Effect of decreased levels of intrinsic tetrahydrobiopterin on endothelial function in anesthetized rats.

  Journal of pharmacological sciences 20080501

• Catalytic reduction of a tetrahydrobiopterin radical within nitric-oxide synthase.

  The Journal of biological chemistry 20080425

• Microvascular function and ageing L-arginine, tetrahydrobiopterin and the search for the fountain of vascular youth.

  The Journal of physiology 20080415

• Chronic treatment with tetrahydrobiopterin reverses endothelial dysfunction and oxidative stress in hypercholesterolaemia.

  Heart (British Cardiac Society) 20080401

• Ratio of 5,6,7,8-tetrahydrobiopterin to 7,8-dihydrobiopterin in endothelial cells determines glucose-elicited changes in NO vs. superoxide production by eNOS.

  American journal of physiology. Heart and circulatory physiology 20080401

• [Mutation analysis and one novel mutation detection of 6-pyruvoyl tetrahydropterin synthase gene in children with tetrahydrobiopterin deficiency].

  Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae 20080401

• Dose dependent effects of reactive oxygen and nitrogen species on the function of neuronal nitric oxide synthase.

  Archives of biochemistry and biophysics 20080315

• Letter by Thum et al regarding article, 'Oxidant stress impairs in vivo reendothelialization capacity of endothelial progenitor cells from patients with type 2 diabetes mellitus: restoration by the peroxisome proliferator-activated receptor-gamma agonist rosiglitazone'.

  Circulation 20080311

• Outcome and long-term follow-up of 36 patients with tetrahydrobiopterin deficiency.

  Molecular genetics and metabolism 20080301

• Endotoxemia-related acute kidney injury in transgenic mice with endothelial overexpression of GTP cyclohydrolase-1.

  American journal of physiology. Renal physiology 20080301

• IGF-I alleviates diabetes-induced RhoA activation, eNOS uncoupling, and myocardial dysfunction.

  American journal of physiology. Regulatory, integrative and comparative physiology 20080301

• Recently-approved sapropterin reduces phenylalanine levels.

  Managed care (Langhorne, Pa.) 20080301

• Endothelium-specific GTP cyclohydrolase I overexpression attenuates blood pressure progression in salt-sensitive low-renin hypertension.

  Circulation 20080226

• Quinones are reduced by 6-tetrahydrobiopterin in human keratinocytes, melanocytes, and melanoma cells.

  Free radical biology & medicine 20080215

• Ageing diminishes endothelium-dependent vasodilatation and tetrahydrobiopterin content in rat skeletal muscle arterioles.

  The Journal of physiology 20080215

• A novel mutation (c.64_65delGGinsAACC [p.G21fsX66]) in the GTP cyclohydrolase 1 gene that causes Segawa disease.

  Journal of neurology, neurosurgery, and psychiatry 20080201

• Classifying tetrahydrobiopterin responsiveness in the hyperphenylalaninaemias.

  Journal of inherited metabolic disease 20080201

• Sapropterin dihydrochloride, 6-R-L-erythro-5,6,7,8-tetrahydrobiopterin, in the treatment of phenylketonuria.

  Expert opinion on investigational drugs 20080201

• Defining tetrahydrobiopterin (BH4)-responsiveness in PKU.

  Journal of inherited metabolic disease 20080201

• Tetrahydrobiopterin.

  Current hypertension reports 20080201

• Over-expression of GTP-cyclohydrolase 1 feedback regulatory protein attenuates LPS and cytokine-stimulated nitric oxide production.

  Vascular medicine (London, England) 20080201

• First drug approved for treatment of phenylketonuria.

  American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists 20080115

• Oxygen-induced radical intermediates in the nNOS oxygenase domain regulated by L-arginine, tetrahydrobiopterin, and thiol.

  Biochemistry 20080108

• A case of 6-pyruvoyl-tetrahydropterin synthase deficiency demonstrates a more significant correlation of L-Dopa dosage with serum prolactin levels than CSF homovanillic acid levels.

  Brain & development 20080101

• Insulin resistance and endothelial cell dysfunction: studies in mammalian models.

  Experimental physiology 20080101

• Molecular genetics of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency.

  Human mutation 20080101

• Oral administration of tetrahydrobiopterin attenuates testicular damage by reducing nitric oxide synthase activity in a cryptorchid mouse model.

  Journal of andrology 20080101

• Preventing effects of a novel anti-parkinsonian agent zonisamide on dopamine quinone formation.

  Neuroscience research 20080101

• Diabetes induces changes of catecholamines in primary mesangial cells.

  The international journal of biochemistry & cell biology 20080101

• Hydrogen peroxide stimulates tetrahydrobiopterin synthesis through activation of the Jak2 tyrosine kinase pathway in vascular endothelial cells.

  The international journal of biochemistry & cell biology 20080101

• Is sapropterin treatment suitable for all subjects with phenylketonuria?

  Expert opinion on pharmacotherapy 20080101

• New drugs: nebivolol hydrochloride, nilotinib, and sapropterin dihydrochloride.

  Journal of the American Pharmacists Association : JAPhA 20080101

• Effects of pioglitazone on nitric oxide bioavailability measured using a catheter-type nitric oxide sensor in angiotensin II-infusion rabbit.

  Hypertension research : official journal of the Japanese Society of Hypertension 20080101

• 2,4-Diamino-6-hydroxypyrimidine (DAHP) suppresses cytokine-induced VCAM-1 expression on the cell surface of human umbilical vein endothelial cells in a BH(4)-independent manner.

  Biochimica et biophysica acta 20080101

• Generalised cranial artery spasm in human rabies.

  Developments in biologicals 20080101

• Systematic evaluation of nitric oxide, tetrahydrobiopterin, and anandamide levels in a porcine model of endotoxemia.

  Journal of anesthesia 20080101

• Exercise training enhanced myocardial endothelial nitric oxide synthase (eNOS) function in diabetic Goto-Kakizaki (GK) rats.

  Cardiovascular diabetology 20080101

• Pharmacokinetics of sapropterin in patients with phenylketonuria.

  Clinical pharmacokinetics 20080101

• Effects of tetrahydrobiopterin on nitric oxide bioavailability and renal hemodynamics in healthy volunteers.

  Journal of nephrology 20080101

• Metabolism of tetrahydrobiopterin: its relevance in monoaminergic neurons and neurological disorders.

  Chemical record (New York, N.Y.) 20080101

• Effects of tetrahydrobiopterin on coronary vascular reactivity in atherosclerotic human coronary arteries.

  Cardiovascular research 20071201

• Effects of tetrahydrobiopterin and phenylalanine on in vivo human phenylalanine hydroxylase by phenylalanine breath test.

  Molecular genetics and metabolism 20071201

• Novel mechanism and role of angiotensin II induced vascular endothelial injury in hypertensive diastolic heart failure.

  Arteriosclerosis, thrombosis, and vascular biology 20071201

• C-reactive protein decreases endothelial nitric oxide synthase activity via uncoupling.

  Journal of molecular and cellular cardiology 20071201

• Endothelium-targeted transgenic GTP-cyclohydrolase I overexpression inhibits neointima formation in mouse carotid artery.

  Clinical and experimental pharmacology & physiology 20071201

• Recommendations for evaluation of responsiveness to tetrahydrobiopterin (BH(4)) in phenylketonuria and its use in treatment.

  Molecular genetics and metabolism 20071201

• Uncoupled endothelial nitric oxide synthase and oxidative stress in a rat model of pregnancy-induced hypertension.

  American journal of hypertension 20071201

• The potential anti-inflammatory effect of tetrahydrobiopterin administration in renal mass reduction-induced chronic renal failure in rats.

  Saudi medical journal 20071201

• Hydrogen sulfide inhibits activity of three isoforms of recombinant nitric oxide synthase.

  Toxicology 20071120

• 4-Hydroxy-2-nonenal increases superoxide anion radical in endothelial cells via stimulated GTP cyclohydrolase proteasomal degradation.

  Arteriosclerosis, thrombosis, and vascular biology 20071101

• Sapropterin dihydrochloride (Kuvan/phenoptin), an orally active synthetic form of BH4 for the treatment of phenylketonuria.

  IDrugs : the investigational drugs journal 20071101

• Oxygenase domain of Drosophila melanogaster nitric oxide synthase: unique kinetic parameters enable a more efficient NO release.

  Biochemistry 20071023

• Regulation of tetrahydrobiopterin biosynthesis by shear stress.

  Circulation research 20071012

• Endothelial cell tetrahydrobiopterin: going with the flow.

  Circulation research 20071012

• The tetrahydrobiopterin loading test in 36 patients with hyperphenylalaninaemia: evaluation of response and subsequent treatment.

  Journal of inherited metabolic disease 20071001

• The response of patients with phenylketonuria and elevated serum phenylalanine to treatment with oral sapropterin dihydrochloride (6R-tetrahydrobiopterin): a phase II, multicentre, open-label, screening study.

  Journal of inherited metabolic disease 20071001

• Oxygen metabolism by endothelial nitric-oxide synthase.

  The Journal of biological chemistry 20070928

• A simple method to calculate the accessible volume of protein-bound ligands: application for ligand selectivity.

  Journal of molecular graphics & modelling 20070901

• Suppressive effect of paroxetine, a selective serotonin uptake inhibitor, on tetrahydrobiopterin levels and dopamine as well as serotonin turnover in the mesoprefrontal system of mice.

  Synapse (New York, N.Y.) 20070901

• [Molecular mechanism of cardiovascular damage induced by aldosterone].

  Yakugaku zasshi : Journal of the Pharmaceutical Society of Japan 20070901

• Therapeutic potential of tetrahydrobiopterin for treating vascular and cardiac disease.

  Journal of cardiovascular pharmacology 20070901

• Proteasome-dependent degradation of guanosine 5'-triphosphate cyclohydrolase I causes tetrahydrobiopterin deficiency in diabetes mellitus.

  Circulation 20070821

• Interaction energies between tetrahydrobiopterin analogues and aromatic residues in tyrosine hydroxylase and phenylalanine hydroxylase.

  The journal of physical chemistry. B 20070816

• Tetrahydrobiopterin for patients with phenylketonuria.

  Lancet (London, England) 20070811

• Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin, 6R-BH4) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomised placebo-controlled study.

  Lancet (London, England) 20070811

• Role of cyclooxygenase-2 in tetrahydrobiopterin-induced dopamine oxidation.

  Biochemical and biophysical research communications 20070803

• Effects and clinical significance of tetrahydrobiopterin supplementation in phenylalanine hydroxylase-deficient hyperphenylalaninaemia.

  Journal of inherited metabolic disease 20070801

• Additive renoprotective effect of candesartan and tetrahydrobiopterin in rats after 5/6 nephrectomy.

  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 20070701

• A specific role for eNOS-derived reactive oxygen species in atherosclerosis progression.

  Arteriosclerosis, thrombosis, and vascular biology 20070701

• Mechanisms for the role of tetrahydrobiopterin in endothelial function and vascular disease.

  Clinical science (London, England : 1979) 20070701

• Nitric oxide accelerates the degradation of tetrahydrobiopterin but not total neopterin in cerebrospinal fluid; potential implications for the assessment of tetrahydrobiopterin metabolism.

  Annals of clinical biochemistry 20070701

• Advances in melanocyte basic science research.

  Dermatologic clinics 20070701

• Resveratrol inhibits rat aortic vascular smooth muscle cell proliferation via estrogen receptor dependent nitric oxide production.

  Journal of cardiovascular pharmacology 20070701

• Metabolic basis of sexual dimorphism in PKU mice after genome-targeted PAH gene therapy.

  Molecular therapy : the journal of the American Society of Gene Therapy 20070601

• (6R)-5,6,7,8-tetrahydro-L-biopterin and its stereoisomer prevent ischemia reperfusion injury in human forearm.

  Arteriosclerosis, thrombosis, and vascular biology 20070601

• Effect of peripherally administered lipopolysaccharide (LPS) on GTP cyclohydrolase I, tetrahydrobiopterin and norepinephrine in the locus coeruleus in mice.

  Stress (Amsterdam, Netherlands) 20070601

• Assessment of tetrahydrobiopterin (BH4) responsiveness in phenylketonuria.

  The Journal of pediatrics 20070601

• Fluvastatin reverses endothelial dysfunction and increased vascular oxidative stress in rat adjuvant-induced arthritis.

  Arthritis and rheumatism 20070601

• [Clinical study of tetrahydrobiopterin responsive phenylalanine hydroxylase deficiency in southern and northern Chinese patients].

  Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 20070601

• Primary role of superoxide anion generation in the cascade of events leading to endothelial dysfunction and damage in high glucose treated HUVEC.

  Nutrition, metabolism, and cardiovascular diseases : NMCD 20070501

• BH4 responsiveness associated to a PKU mutation with decreased binding affinity for the cofactor.

  Clinica chimica acta; international journal of clinical chemistry 20070501

• Early initiation of L-dopa therapy enables stable development of executive function in tetrahydrobiopterin (BH4) deficiency.

  Developmental medicine and child neurology 20070501

• Nitric oxide modulates expression of extracellular matrix genes linked to fibrosis in kidney mesangial cells.

  Biological chemistry 20070501

• Tetrahydrobiopterin availability, nitric oxide metabolism and glutathione status in the hph-1 mouse; implications for the pathogenesis and treatment of tetrahydrobiopterin deficiency states.

  Journal of inherited metabolic disease 20070401

• Protective effect of sulforaphane against dopaminergic cell death.

  The Journal of pharmacology and experimental therapeutics 20070401

• Role of the multidrug resistance protein-1 in hypertension and vascular dysfunction caused by angiotensin II.

  Arteriosclerosis, thrombosis, and vascular biology 20070401

• Oral administration of tetrahydrobiopterin slows the progression of atherosclerosis in apolipoprotein E-knockout mice.

  Arteriosclerosis, thrombosis, and vascular biology 20070401

• Phenylketonuria: dietary and therapeutic challenges.

  Journal of inherited metabolic disease 20070401

• Regulation of GTP cyclohydrolase I expression by orphan receptor Nurr1 in cell culture and in vivo.

  Journal of neurochemistry 20070401

• [Screening for tetrahydrobiopterin metabolic disorders and related gene analysis among the patients with motor disturbance and mental retardation].

  Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 20070401

• Sepiapterin reductase expression is increased in Parkinson's disease brain tissue.

  Brain research 20070330

• Intracortical inhibition of the motor cortex in Segawa disease (DYT5).

  Neurology 20070327

• Interaction of genetic and environmental factors in a Drosophila parkinsonism model.

  The Journal of neuroscience : the official journal of the Society for Neuroscience 20070307

• Molecular genetics of tetrahydrobiopterin (BH4) deficiency in the Maltese population.

  Molecular genetics and metabolism 20070301

• Quantitation of total biopterin and tetrahydrobiopterin in plasma.

  Clinical biochemistry 20070301

• Folic acid prevents and partially reverses glucocorticoid-induced hypertension in the rat.

  American journal of hypertension 20070301

• Endothelial nitric oxide synthase uncoupling impairs endothelial progenitor cell mobilization and function in diabetes.

  Diabetes 20070301

• [Molecular imaging for drug development].

  Brain and nerve = Shinkei kenkyu no shinpo 20070301

• Oral administration of both tetrahydrobiopterin and L-arginine prevents endothelial dysfunction in rats with chronic renal failure.

  Journal of cardiovascular pharmacology 20070301

• Insulin enhances nitric oxide production in trabecular meshwork cells via de novo pathway for tetrahydrobiopterin synthesis.

  Korean journal of ophthalmology : KJO 20070301

• Buthionine sulfoximine causes endothelium dependent hyper-relaxation and hypoadiponectinemia.

  Life sciences 20070206

• Oxidative stress via hydrogen peroxide affects proopiomelanocortin peptides directly in the epidermis of patients with vitiligo.

  The Journal of investigative dermatology 20070201

• 17beta-estradiol antagonizes the down-regulation of endothelial nitric-oxide synthase and GTP cyclohydrolase I by high glucose: relevance to postmenopausal diabetic cardiovascular disease.

  The Journal of pharmacology and experimental therapeutics 20070201

• Tetrahydrobiopterin prevents endothelial dysfunction and restores adiponectin levels in rats.

  European journal of pharmacology 20070119

• Relationships between nitric oxide-mediated endothelial function, eNOS coupling and blood pressure revealed by eNOS-GTP cyclohydrolase 1 double transgenic mice.

  Experimental physiology 20070101

• Particular vulnerability of rat mesencephalic dopaminergic neurons to tetrahydrobiopterin: Relevance to Parkinson's disease.

  Neurobiology of disease 20070101

• Ribavirin inhibits angiogenesis by tetrahydrobiopterin depletion.

  FASEB journal : official publication of the Federation of American Societies for Experimental Biology 20070101

• Analysis of plasma biopterin levels in psychiatric disorders suggests a common BH4 deficit in schizophrenia and schizoaffective disorder.

  Neurochemical research 20070101

• Tetrahydrobiopterin availability in Parkinson's and Alzheimer's disease; potential pathogenic mechanisms.

  Neurochemical research 20070101

• Chemiluminescence assay for tetrahydrobiopterin based on the generation of hydrogen peroxide using isoluminol-microperoxidase in the presence of 1-methoxy PMS.

  Luminescence : the journal of biological and chemical luminescence 20070101

• Effects of supplementation of BH4 after prolonged ischemia in skeletal muscle.

  Microsurgery 20070101

• C-Reactive protein-induced endothelial microparticle generation in HUVECs is related to BH4-dependent NO formation.

  Journal of vascular research 20070101

• Assessment of tetrahydrobiopterin responsiveness in Turkish hyperphenylalaninemic patients.

  The Turkish journal of pediatrics 20070101

• Mutations in the regulatory domain of phenylalanine hydroxylase and response to tetrahydrobiopterin.

  Genetic testing 20070101

• Paradoxical effects of two oximes on nitric oxide production by purified NO synthases, in cell culture and in animals.

  Nitric oxide : biology and chemistry 20070101

• Glucocorticoids and oxidative stress.

  Journal of basic and clinical physiology and pharmacology 20070101

• Changes of asymmetric dimethylarginine, nitric oxide, tetrahydrobiopterin, and oxidative stress in patients with acute myocardial infarction by medical treatments.

  Clinical hemorheology and microcirculation 20070101

• Gamma-tocopherol may promote effective no synthase function by protecting tetrahydrobiopterin from peroxynitrite.

  Medical hypotheses 20070101

• Biochemistry of postmortem brains in Parkinson's disease: historical overview and future prospects.

  Journal of neural transmission. Supplementum 20070101

• Plasma pterins and folate in late life depression: the Rotterdam Study.

  Psychiatry research 20061207

• Pharmacokinetics of orally administered tetrahydrobiopterin in patients with phenylalanine hydroxylase deficiency.

  Journal of inherited metabolic disease 20061201

• [Detection of the prevalent mutations of 6-pyruvoyl-tetrahydropterin synthase gene by PCR-RFLP analysis in Chinese patients].

  Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 20061201

• Electronic structure, ionization potential, and electron affinity of the enzyme cofactor (6R)-5,6,7,8-tetrahydrobiopterin in the gas phase, solution, and protein environments.

  The journal of physical chemistry. B 20061116

• Prediction of estrogen receptor agonists and characterization of associated molecular descriptors by statistical learning methods.

  Journal of molecular graphics & modelling 20061101

• GTP cyclohydrolase feedback regulatory protein controls cofactor 6-tetrahydrobiopterin synthesis in the cytosol and in the nucleus of epidermal keratinocytes and melanocytes.

  The Journal of investigative dermatology 20061101

• Tetrahydrobiopterin and cardiovascular disease.

  Arteriosclerosis, thrombosis, and vascular biology 20061101

• GTP cyclohydrolase and tetrahydrobiopterin regulate pain sensitivity and persistence.

  Nature medicine 20061101

• Feeling pain? Who's your daddy...

  Nature medicine 20061101

• Self-assembly of the 2-His-1-carboxylate facial triad in mononuclear iron(II) and zinc(II) models of metalloenzyme active sites.

  Inorganic chemistry 20061002

• Specific interaction of the diastereomers 7(R)- and 7(S)-tetrahydrobiopterin with phenylalanine hydroxylase: implications for understanding primapterinuria and vitiligo.

  FASEB journal : official publication of the Federation of American Societies for Experimental Biology 20061001

• 5-methyltetrahydrofolate rapidly improves endothelial function and decreases superoxide production in human vessels: effects on vascular tetrahydrobiopterin availability and endothelial nitric oxide synthase coupling.

  Circulation 20060912

• The role of tetrahydrobiopterin in catalysis by nitric oxide synthase.

  Chemical communications (Cambridge, England) 20060907

• 6-Pyruvoyltetrahydropterin synthase orthologs of either a single or dual domain structure are responsible for tetrahydrobiopterin synthesis in bacteria.

  FEBS letters 20060904

• Tetrahydrobiopterin protects against guanabenz-mediated inhibition of neuronal nitric-oxide synthase in vitro and in vivo.

  Drug metabolism and disposition: the biological fate of chemicals 20060901

• Systemic oxidative stress associated with lung resection during single lung ventilation.

  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 20060901

• Effect of tetrahydrobiopterin on selective endothelial dysfunction of epicardial porcine coronary arteries induced by cardiopulmonary bypass.

  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 20060901

• Early determinants of H2O2-induced endothelial dysfunction.

  Free radical biology & medicine 20060901

• Mutations in the BH4-metabolizing genes GTP cyclohydrolase I, 6-pyruvoyl-tetrahydropterin synthase, sepiapterin reductase, carbinolamine-4a-dehydratase, and dihydropteridine reductase.

  Human mutation 20060901

• Three-year audit of the hyperphenylalaninaemia/phenylketonuria spectrum in Victoria.

  Journal of paediatrics and child health 20060901

• Decrease in tetrahydrobiopterin as a possible cause of nephropathy in type II diabetic rats.

  Kidney international 20060801

• Pigment pattern formation in the quail mutant of the silkworm, Bombyx mori: parallel increase of pteridine biosynthesis and pigmentation of melanin and ommochromes.

  Pigment cell research 20060801

• Inefficient spin trapping of superoxide in the presence of nitric-oxide: implications for studies on nitric-oxide synthase uncoupling.

  Free radical biology & medicine 20060801

• Ascorbic acid synthesis due to L-gulono-1,4-lactone oxidase expression enhances NO production in endothelial cells.

  Biochemical and biophysical research communications 20060714

• Tetrahydrobiopterin stimulates L-DOPA release from striatal tissue.

  European journal of pharmacology 20060710

• eNOS gene therapy exacerbates hepatic ischemia-reperfusion injury in diabetes: a role for eNOS uncoupling.

  Circulation research 20060707

• Manganese modulates pro-inflammatory gene expression in activated glia.

  Neurochemistry international 20060701

• Endothelial NO synthase target of aldosterone.

  Hypertension (Dallas, Tex. : 1979) 20060701

• Molecular mechanism of the inhibitory effect of aldosterone on endothelial NO synthase activity.

  Hypertension (Dallas, Tex. : 1979) 20060701

• The eNOS cofactor tetrahydrobiopterin improves endothelial dysfunction in livers of rats with CCl4 cirrhosis.

  Hepatology (Baltimore, Md.) 20060701

• Tetrahydrobiopterin attenuates microvascular reperfusion injury following murine pancreas transplantation.

  American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 20060701

• Tetrahydrobiopterin improves aging-related impairment of endothelium-dependent vasodilation through increase in nitric oxide production.

  Atherosclerosis 20060601

• Cytokine-induced metabolic effects in human adipocytes are independent of endogenous nitric oxide.

  American journal of physiology. Endocrinology and metabolism 20060601

• Increased vascular biosynthesis of tetrahydrobiopterin in apolipoprotein E-deficient mice.

  American journal of physiology. Heart and circulatory physiology 20060601

• Regulation of inducible nitric oxide synthase in post-operative adhesions.

  Human reproduction (Oxford, England) 20060601

• A yeast 2-hybrid analysis of human GTP cyclohydrolase I protein interactions.

  Journal of neurochemistry 20060601

• Endothelial cell superoxide anion radical generation is not dependent on endothelial nitric oxide synthase-serine 1179 phosphorylation and endothelial nitric oxide synthase dimer/monomer distribution.

  Free radical biology & medicine 20060601

• Endothelial dysfunction in rat adjuvant-induced arthritis: vascular superoxide production by NAD(P)H oxidase and uncoupled endothelial nitric oxide synthase.

  Arthritis and rheumatism 20060601

• [Study on tetrahydrobiopterin deficiency in Northern Chinese population].

  Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 20060601

• Catalase protects cardiomyocytes via its inhibition of nitric oxide synthesis.

  Nitric oxide : biology and chemistry 20060501

• A murine model for human sepiapterin-reductase deficiency.

  American journal of human genetics 20060401

• Circadian rhythms in RLS.

  Sleep medicine 20060401

• Evaluation of electrospray-tandem mass spectrometry for the detection of phenylketonuria and other rare disorders.

  Molecular nutrition & food research 20060401

• Molecular mechanism for pterin-mediated inactivation of tyrosine hydroxylase: formation of insoluble aggregates of tyrosine hydroxylase.

  Journal of biochemistry 20060401

• Cardioprotective effects of tetrahydrobiopterin in cold heart preservation after cardiac arrest.

  Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia 20060401

• Regulation of the monomer-dimer equilibrium in inducible nitric-oxide synthase by nitric oxide.

  The Journal of biological chemistry 20060324

• Analysis of the effect of tetrahydrobiopterin on PAH gene expression in hepatoma cells.

  FEBS letters 20060320

• Administration of secretin for autism alters dopamine metabolism in the central nervous system.

  Brain & development 20060301

• Functional identification of orthologous genes encoding pterin recycling activity in Plasmodium falciparum and Toxoplasma gondii.

  Molecular and biochemical parasitology 20060301

• Tetrahydrobiopterin causes mitochondrial dysfunction in dopaminergic cells: implications for Parkinson's disease.

  Neurochemistry international 20060301

• High-pressure studies of the reaction mechanism of nitric-oxide synthase.

  Biochimica et biophysica acta 20060301

• Dissociation and unfolding of inducible nitric oxide synthase oxygenase domain identifies structural role of tetrahydrobiopterin in modulating the heme environment.

  Molecular and cellular biochemistry 20060301

• Evaluation of tetrahydrobiopterin (BH4) as a potential therapeutic agent to treat erectile dysfunction.

  Asian journal of andrology 20060301

• Cytoprotective function of tetrahydrobiopterin in rat liver ischemia/reperfusion injury.

  Surgery 20060301

• Tetrahydrobiopterin compounds prolong allograft survival independently of their effect on nitric oxide synthase activity.

  Transplantation 20060227

• Reduction and oxidation of the active site iron in tyrosine hydroxylase: kinetics and specificity.

  Biochemistry 20060221

• eNOS function is developmentally regulated: uncoupling of eNOS occurs postnatally.

  American journal of physiology. Lung cellular and molecular physiology 20060201

• Increased blood flow causes coordinated upregulation of arterial eNOS and biosynthesis of tetrahydrobiopterin.

  American journal of physiology. Heart and circulatory physiology 20060201

• Long-term follow-up of Chinese patients who received delayed treatment for 6-pyruvoyl-tetrahydropterin synthase deficiency.

  Molecular genetics and metabolism 20060201

• Reduction of myocardial infarct size by tetrahydrobiopterin: possible involvement of mitochondrial KATP channels activation through nitric oxide production.

  Journal of cardiovascular pharmacology 20060201

• [Tetrahydrobiopterin therapy for hyperphenylalaninemia due to phenylalanine hydroxylase deficiency. When and how?].

  Anales de pediatria (Barcelona, Spain : 2003) 20060201

• The spectrum of phenylalanine variations under tetrahydrobiopterin load in subjects affected by phenylalanine hydroxylase deficiency.

  Journal of inherited metabolic disease 20060201

• Structure of Chlorobium tepidum sepiapterin reductase complex reveals the novel substrate binding mode for stereospecific production of L-threo-tetrahydrobiopterin.

  The Journal of biological chemistry 20060127

• Folic acid reverses endothelial dysfunction induced by inhibition of tetrahydrobiopterin biosynthesis.

  European journal of pharmacology 20060120

• Late developmental stage-specific role of tryptophan hydroxylase 1 in brain serotonin levels.

  The Journal of neuroscience : the official journal of the Society for Neuroscience 20060111

• A burning issue: do sepsis and systemic inflammatory response syndrome (SIRS) directly contribute to cardiac dysfunction?

  Frontiers in bioscience : a journal and virtual library 20060101

• Neural tube closure depends on nitric oxide synthase activity.

  Journal of neurochemistry 20060101

• Effect of 5-lipoxygenase blockade on blood pressure and acetylcholine-evoked endothelium-dependent contraction in aorta from spontaneously hypertensive rats.

  Journal of hypertension 20060101

• Tetrahydrobiopterin, but not L-arginine, decreases NO synthase uncoupling in cells expressing high levels of endothelial NO synthase.

  Hypertension (Dallas, Tex. : 1979) 20060101

• Reactivity of the heme-dioxygen complex of the inducible nitric oxide synthase in the presence of alternative substrates.

  The FEBS journal 20060101

• [Dopa-responsive dystonia: clinical, genetic, and biochemical studies].

  Rinsho shinkeigaku = Clinical neurology 20060101

• Estrogen therapy replenishes vascular tetrahydrobiopterin and reduces oxidative stress in ovariectomized rats.

  Menopause (New York, N.Y.) 20060101

• Correction of endothelial dysfunction in diabetic female rats by tetrahydrobiopterin and chronic insulin.

  Journal of vascular research 20060101

• Activation energy determinations suggest that thiols reverse autooxidation of tetrahydrobiopterin by a different mechanism than ascorbate.

  The international journal of biochemistry & cell biology 20060101

• Protection of endothelial function: targets for nutritional and pharmacological interventions.

  Journal of cardiovascular pharmacology 20060101

• Nitric oxide and neopterin in bipolar affective disorder.

  Neuropsychobiology 20060101

• Novel therapies targeting vascular endothelium.

  Endothelium : journal of endothelial cell research 20060101

• Tetrahydrobiopterin depletion and ubiquitylation of neuronal nitric oxide synthase.

  Brain research. Molecular brain research 20051207

• The activity of wild-type and mutant phenylalanine hydroxylase and its regulation by phenylalanine and tetrahydrobiopterin at physiological and pathological concentrations: an isothermal titration calorimetry study.

  Molecular genetics and metabolism 20051201

• Clinical and nutritional evaluation of phenylketonuric patients on tetrahydrobiopterin monotherapy.

  Molecular genetics and metabolism 20051201

• Long-term treatment with tetrahydrobiopterin increases phenylalanine tolerance in children with severe phenotype of phenylketonuria.

  Molecular genetics and metabolism 20051201

• Kinetic and stability analysis of PKU mutations identified in BH4-responsive patients.

  Molecular genetics and metabolism 20051201

• Tetrahydrobiopterin-responsive phenylketonuria: the New South Wales experience.

  Molecular genetics and metabolism 20051201

• Response of patients with phenylketonuria in the US to tetrahydrobiopterin.

  Molecular genetics and metabolism 20051201

• Accumulated BH4 in mouse liver caused by administration of either 6R- or 6SBH4 consisted solely of the 6R-diastereomer: evidence of oxidation to BH2 and enzymic reduction.

  Molecular genetics and metabolism 20051201

• Spanish BH4-responsive phenylalanine hydroxylase-deficient patients: evolution of seven patients on long-term treatment with tetrahydrobiopterin.

  Molecular genetics and metabolism 20051201

• Cellular accumulation of tetrahydrobiopterin following its administration is mediated by two different processes; direct uptake and indirect uptake mediated by a methotrexate-sensitive process.

  Molecular genetics and metabolism 20051201

• Neonatal hyperphenylalaninemia, perinatal hemochromatosis, and renal tubulopathy: a unique patient or a novel metabolic disorder?

  Molecular genetics and metabolism 20051201

• Plasma biopterin levels and tetrahydrobiopterin responsiveness.

  Molecular genetics and metabolism 20051201

• Incidence of BH4-responsiveness in phenylalanine-hydroxylase-deficient Italian patients.

  Molecular genetics and metabolism 20051201

• Hyperhomocystinemia impairs endothelial function and eNOS activity via PKC activation.

  Arteriosclerosis, thrombosis, and vascular biology 20051201

• Long-term treatment of patients with mild and classical phenylketonuria by tetrahydrobiopterin.

  Molecular genetics and metabolism 20051201

• Delivery of exogenous tetrahydrobiopterin (BH4) to cells of target organs: role of salvage pathway and uptake of its precursor in effective elevation of tissue BH4.

  Molecular genetics and metabolism 20051201

• Screening for tetrahydrobiopterin deficiencies using dried blood spots on filter paper.

  Molecular genetics and metabolism 20051201

• Inhibition of brain GTP cyclohydrolase I and tetrahydrobiopterin attenuates cerebral infarction via reducing inducible NO synthase and peroxynitrite in ischemic stroke.

  Stroke 20051201

• Extended tetrahydrobiopterin loading test in the diagnosis of cofactor-responsive phenylketonuria: a pilot study.

  Molecular genetics and metabolism 20051201

• Stimulation of hepatic phenylalanine hydroxylase activity but not Pah-mRNA expression upon oral loading of tetrahydrobiopterin in normal mice.

  Molecular genetics and metabolism 20051201

• Tetrahydrobiopterin and maternal PKU.

  Molecular genetics and metabolism 20051201

• Exploring the redox reactions between heme and tetrahydrobiopterin in the nitric oxide synthases.

  Dalton transactions (Cambridge, England : 2003) 20051107

• Genetically rescued tetrahydrobiopterin-depleted mice survive with hyperphenylalaninemia and region-specific monoaminergic abnormalities.

  Journal of neurochemistry 20051101

• Tetrahydrobiopterin augments endothelium-dependent dilatation in sedentary but not in habitually exercising older adults.

  The Journal of physiology 20051101

• Stoichiometric relationships between endothelial tetrahydrobiopterin, endothelial NO synthase (eNOS) activity, and eNOS coupling in vivo: insights from transgenic mice with endothelial-targeted GTP cyclohydrolase 1 and eNOS overexpression.

  Circulation research 20051028

• Sepiapterin attenuates 1-methyl-4-phenylpyridinium-induced apoptosis in neuroblastoma cells transfected with neuronal NOS: role of tetrahydrobiopterin, nitric oxide, and proteasome activation.

  Free radical biology & medicine 20051015

• Hypercholesterolemia abrogates late preconditioning via a tetrahydrobiopterin-dependent mechanism in conscious rabbits.

  Circulation 20051004

• Tetrahydrobiopterin corrects Escherichia coli endotoxin-induced endothelial dysfunction.

  American journal of physiology. Heart and circulatory physiology 20051001

• Roles of Ca2+ and the Ca2+-sensing receptor (CASR) in the expression of inducible NOS (nitric oxide synthase)-2 and its BH4 (tetrahydrobiopterin)-dependent activation in cytokine-stimulated adult human astrocytes.

  Journal of cellular biochemistry 20051001

• Supplement of tetrahydrobiopterin by a gene transfer of GTP cyclohydrolase I cDNA improves vascular dysfunction in insulin-resistant rats.

  Journal of cardiovascular pharmacology 20051001

• Tetrahydrobiopterin in the treatment of children with autistic disorder: a double-blind placebo-controlled crossover study.

  Journal of clinical psychopharmacology 20051001

• Immobilization stress causes increases in tetrahydrobiopterin, dopamine, and neuromelanin and oxidative damage in the nigrostriatal system.

  Journal of neurochemistry 20051001

• Endothelial nitric oxide synthase dysfunction in diabetic mice: importance of tetrahydrobiopterin in eNOS dimerisation.

  Diabetologia 20050901

• Tetrahydrobiopterin deficiency exaggerates intimal hyperplasia after vascular injury.

  American journal of physiology. Regulatory, integrative and comparative physiology 20050801

• An oxygraphic method for determining kinetic properties and catalytic mechanism of aromatic amino acid hydroxylases.

  Analytical biochemistry 20050801

• [Tetrahydrobiopterin loading test in differential diagnosis among hyperphenylalaninemia patients].

  Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 20050801

• Structural analysis of isoform-specific inhibitors targeting the tetrahydrobiopterin binding site of human nitric oxide synthases.

  Journal of medicinal chemistry 20050728

• Syntheses of NAMDA derivatives inhibiting NO production in BV-2 cells stimulated with lipopolysaccharide.

  Bioorganic & medicinal chemistry letters 20050715

• Correction of endothelial dysfunction by tetrahydrobiopterin: new hope for the treatment of arterial hypertension?

  Journal of hypertension 20050701

• Supplementation with tetrahydrobiopterin prevents the cardiovascular effects of angiotensin II-induced oxidative and nitrosative stress.

  Journal of hypertension 20050701

• 6-pyruvoyl-tetrahydropterin synthase deficiency with mild hyperphenylalaninemia.

  Annals of neurology 20050701

• NAD(P)H oxidase and uncoupled nitric oxide synthase are major sources of glomerular superoxide in rats with experimental diabetic nephropathy.

  American journal of physiology. Renal physiology 20050601

• Severe mucitis after sublingual administration of tetrahydrobiopterin in a patient with tetrahydrobiopterin-responsive phenylketonuria.

  European journal of pediatrics 20050601

• Angiotensin-II-induced oxidative stress elicits hypoadiponectinaemia in rats.

  Diabetologia 20050601

• Long-term glial cell line-derived neurotrophic factor overexpression in the intact nigrostriatal system in rats leads to a decrease of dopamine and increase of tetrahydrobiopterin production.

  Journal of neurochemistry 20050601

• Cyclosporine-induced coronary endothelial dysfunction: is tetrahydrobiopterin the solution?

  Transplantation proceedings 20050601

• Tetrahydrobiopterin and nitric oxide synthase dimer levels are not changed following hypoxia-ischemia in the newborn rat.

  Brain research. Developmental brain research 20050512

• Tetrahydrobiopterin prevents platelet-activating factor-induced intestinal hypoperfusion and necrosis: Role of neuronal nitric oxide synthase.

  Critical care medicine 20050501

• Reductive activation of Cr(Vi) by nitric oxide synthase.

  Chemical research in toxicology 20050501

• [Screening and diagnosis of tetrahydrobiopterin responsive phenylalanine hydroxylase deficiency with tetrahydrobiopterin loading test].

  Zhonghua er ke za zhi = Chinese journal of pediatrics 20050501

• Tetrahydrobiopterin prevents cyclosporine-induced vasomotor dysfunction.

  Transplantation 20050427

• Pulmonary hypertension in a GTP-cyclohydrolase 1-deficient mouse.

  Circulation 20050426

• Pivotal role for endothelial tetrahydrobiopterin in pulmonary hypertension.

  Circulation 20050426

• Tetrahydrobiopterin in pulmonary hypertension: pulmonary hypertension in guanosine triphosphate-cyclohydrolase-deficient mice.

  Circulation 20050426

• Regulation of tyrosinase by tetrahydropteridines--what is real? A comment on the work published by Wood et al. on December 24, 2004.

  Biochemical and biophysical research communications 20050415

• Possible involvement of hydroxyl radical on the stimulation of tetrahydrobiopterin synthesis by hydrogen peroxide and peroxynitrite in vascular endothelial cells.

  The international journal of biochemistry & cell biology 20050401

• A tryptophan that modulates tetrahydrobiopterin-dependent electron transfer in nitric oxide synthase regulates enzyme catalysis by additional mechanisms.

  Biochemistry 20050329

• Regulation of gene expression by lithium and depletion of inositol in slices of adult rat cortex.

  Neuron 20050324

• Nitric oxide dynamics and endothelial dysfunction in type II model of genetic diabetes.

  European journal of pharmacology 20050321

• The three nitric-oxide synthases differ in their kinetics of tetrahydrobiopterin radical formation, heme-dioxy reduction, and arginine hydroxylation.

  The Journal of biological chemistry 20050311

• Endogenous methylarginines modulate superoxide as well as nitric oxide generation from neuronal nitric-oxide synthase: differences in the effects of monomethyl- and dimethylarginines in the presence and absence of tetrahydrobiopterin.

  The Journal of biological chemistry 20050304

• The hyperbilirubinemic Gunn rat is resistant to the pressor effects of angiotensin II.

  American journal of physiology. Renal physiology 20050301

• BH(4) (tetrahydrobiopterin)-dependent activation, but not the expression, of inducible NOS (nitric oxide synthase)-2 in proinflammatory cytokine-stimulated, cultured normal human astrocytes is mediated by MEK-ERK kinases.

  Journal of cellular biochemistry 20050301

• Treatment with tetrahydrobiopterin reduces blood pressure in male SHR by reducing testosterone synthesis.

  American journal of physiology. Regulatory, integrative and comparative physiology 20050301

• Inhibition of vesicular monoamine transporter enhances vulnerability of dopaminergic cells: relevance to Parkinson's disease.

  Neurochemistry international 20050301

• Tetrahydrobiopterin and eNOS dimer/monomer ratio--a clue to eNOS uncoupling in diabetes?

  Cardiovascular research 20050301

• Augmented BH4 by gene transfer restores nitric oxide synthase function in hyperglycemic human endothelial cells.

  Cardiovascular research 20050301

• Sex difference in nitric oxide synthase-dependent dilatation of cerebral arterioles during long-term alcohol consumption.

  Alcoholism, clinical and experimental research 20050301

• Nitric oxide: a major determinant of mast cell phenotype and function.

  Memorias do Instituto Oswaldo Cruz 20050301

• Changes in tetrahydrobiopterin levels in endothelial cells and adult cardiomyocytes induced by LPS and hydrogen peroxide--a role for GFRP?

  Free radical biology & medicine 20050215

• Cytokine-stimulated GTP cyclohydrolase I expression in endothelial cells requires coordinated activation of nuclear factor-kappaB and Stat1/Stat3.

  Circulation research 20050204

• Transcribing the cross-talk of cytokine-induced tetrahydrobiopterin synthesis in endothelial cells.

  Circulation research 20050204

• Exogenous NO suppresses flow-induced endothelium-derived NO production because of depletion of tetrahydrobiopterin.

  American journal of physiology. Heart and circulatory physiology 20050201

• Somatodendritic autoreceptor regulation of serotonergic neurons: dependence on L-tryptophan and tryptophan hydroxylase-activating kinases.

  The European journal of neuroscience 20050201

• Esrom, an ortholog of PAM (protein associated with c-myc), regulates pteridine synthesis in the zebrafish.

  Developmental biology 20050115

• Fluvoxamine, a selective serotonin reuptake inhibitor, suppresses tetrahydrobiopterin levels and dopamine as well as serotonin turnover in the mesoprefrontal system of mice.

  Psychopharmacology 20050101

• Tetrahydrobiopterin restores impaired coronary microvascular dysfunction in hypercholesterolaemia.

  European journal of nuclear medicine and molecular imaging 20050101

• Time-dependent inhibition and tetrahydrobiopterin depletion of endothelial nitric-oxide synthase caused by cigarettes.

  Drug metabolism and disposition: the biological fate of chemicals 20050101

• Sepiapterin reductases from Chlorobium tepidum and Chlorobium limicola catalyze the synthesis of L-threo-tetrahydrobiopterin from 6-pyruvoyltetrahydropterin.

  FEMS microbiology letters 20050101

• Estrogen modifies stress response of catecholamine biosynthetic enzyme genes and cardiovascular system in ovariectomized female rats.

  Neuroscience 20050101

• Beneficial effect of tetrahydrobiopterin on the survival of rats exposed to hepatic ischemia-reperfusion injury.

  Transplantation proceedings 20050101

• Systemic nitric oxide production rate during hemodialysis and its relationship with nitric oxide-related factors.

  Blood purification 20050101

• Evidence for a tetrahydrobiopterin deficit in schizophrenia.

  Neuropsychobiology 20050101

• Tetrahydrobiopterin as combined electron/proton donor in nitric oxide biosynthesis: cryogenic UV-Vis and EPR detection of reaction intermediates.

  Methods in enzymology 20050101

• Platelet serotonin concentrations in PKU patients under dietary control and tetrahydrobiopterin treatment.

  Journal of inherited metabolic disease 20050101

• Regulation of tyrosinase by tetrahydropteridines and H2O2.

  Biochemical and biophysical research communications 20041224

• Mechanisms of aging-induced impairment of endothelium-dependent relaxation: role of tetrahydrobiopterin.

  American journal of physiology. Heart and circulatory physiology 20041201

• GTP cyclohydrolase I gene transfer reverses tetrahydrobiopterin deficiency and increases nitric oxide synthesis in endothelial cells and isolated vessels from diabetic rats.

  FASEB journal : official publication of the Federation of American Societies for Experimental Biology 20041201

• Tetrahydrobiopterin responsiveness in patients with phenylketonuria.

  Clinical biochemistry 20041201

• Malfunction of vascular control in lifestyle-related diseases: endothelial nitric oxide (NO) synthase/NO system in atherosclerosis.

  Journal of pharmacological sciences 20041201

• Evidence of cellular immune activation in children with opsoclonus-myoclonus: cerebrospinal fluid neopterin.

  Journal of child neurology 20041201

• Alpha-Tocopherol and endothelial nitric oxide synthesis.

  Annals of the New York Academy of Sciences 20041201

• Tetrahydrobiopterin protects phenylalanine hydroxylase activity in vivo: implications for tetrahydrobiopterin-responsive hyperphenylalaninemia.

  FEBS letters 20041119

• Tetrahydrobiopterin binding to aromatic amino acid hydroxylases. Ligand recognition and specificity.

  Journal of medicinal chemistry 20041118

• The effect of high glucose on NO and O2- through endothelial GTPCH1 and NADPH oxidase.

  Life sciences 20041112

• Gene expression profiling of inflamed human endothelial cells and influence of activated protein C.

  Circulation 20041102

• Tetrahydrobiopterin increases insulin sensitivity in patients with type 2 diabetes and coronary heart disease.

  American journal of physiology. Endocrinology and metabolism 20041101

• In vivo studies of phenylalanine hydroxylase by phenylalanine breath test: diagnosis of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency.

  Pediatric research 20041101

• Mechanisms underlying responsiveness to tetrahydrobiopterin in mild phenylketonuria mutations.

  Human mutation 20041101

• Tetrahydrobiopterin: regulator of endothelial nitric oxide synthase in vascular disease.

  Trends in cardiovascular medicine 20041101

• Thermodynamic characterization of the binding of tetrahydropterins to phenylalanine hydroxylase.

  Journal of the American Chemical Society 20041027

• Redox function of tetrahydrobiopterin and effect of L-arginine on oxygen binding in endothelial nitric oxide synthase.

  Biochemistry 20041019

• Superoxide dismutase and catalase are required to detect (.-)NO from both coupled and uncoupled neuronal no synthase.

  Free radical biology & medicine 20041001

• Reversible parkinsonism following heroin pyrolysate inhalation is associated with tetrahydrobiopterin deficiency.

  Movement disorders : official journal of the Movement Disorder Society 20041001

• Tetrahydrobiopterin uptake in supplemental administration: elevation of tissue tetrahydrobiopterin in mice following uptake of the exogenously oxidized product 7,8-dihydrobiopterin and subsequent reduction by an anti-folate-sensitive process.

  Journal of pharmacological sciences 20041001

• Substrate regulation of serotonin and dopamine synthesis in Drosophila.

  Invertebrate neuroscience : IN 20041001

• Diffusion tensor images in children with early-treated, chronic, malignant phenylketonuric: correlation with intelligence assessment.

  AJNR. American journal of neuroradiology 20041001

• Biology and chemistry of the inhibition of nitric oxide synthases by pteridine-derivatives as therapeutic agents.

  Medicinal research reviews 20040901

• Reduced vascular NO bioavailability in diabetes increases platelet activation in vivo.

  Arteriosclerosis, thrombosis, and vascular biology 20040901

• Effect of chronic tetrahydrobiopterin supplementation on blood pressure and proteinuria in 5/6 nephrectomized rats.

  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 20040901

• Alpha-tocopherol amplifies phosphorylation of endothelial nitric oxide synthase at serine 1177 and its short-chain derivative trolox stabilizes tetrahydrobiopterin.

  Free radical biology & medicine 20040901

• Comparison of tetrahydrobiopterin and L-arginine on cerebral blood flow after controlled cortical impact injury in rats.

  Journal of neurotrauma 20040901

• [Mechanisms of nitric oxide production].

  Nihon rinsho. Japanese journal of clinical medicine 20040901

• [Diabetic macroangiopathy and NO].

  Nihon rinsho. Japanese journal of clinical medicine 20040901

• The effect of insufficiency of tetrahydrobiopterin on endothelial function and vasoactivity.

  The Japanese journal of thoracic and cardiovascular surgery : official publication of the Japanese Association for Thoracic Surgery = Nihon Kyobu Geka Gakkai zasshi 20040901

• Microvascular dysfunction after transient high glucose is caused by superoxide-dependent reduction in the bioavailability of NO and BH(4).

  American journal of physiology. Heart and circulatory physiology 20040801

• Tetrahydrobiopterin rapidly reduces the SOD mimic Mn(III) ortho-tetrakis(N-ethylpyridinium-2-yl)porphyrin.

  Free radical biology & medicine 20040801

• Tetrahydropteridines suppress gene expression and induce apoptosis of activated RAW264.7 cells via formation of hydrogen peroxide.

  Free radical biology & medicine 20040801

• Trends in enzyme therapy for phenylketonuria.

  Molecular therapy : the journal of the American Society of Gene Therapy 20040801

• Three different oxygen-induced radical species in endothelial nitric-oxide synthase oxygenase domain under regulation by L-arginine and tetrahydrobiopterin.

  The Journal of biological chemistry 20040730

• Response of tyrosine hydroxylase and GTP cyclohydrolase I gene expression to estrogen in brain catecholaminergic regions varies with mode of administration.

  Brain research 20040723

• Decreased platelet nitric oxide synthase activity and plasma nitric oxide metabolites in major depressive disorder.

  Biological psychiatry 20040715

• Functional tetrahydrobiopterin synthesis in human platelets.

  Circulation 20040713

• Spectrophotometric analysis of the protective effect of ascorbate against spontaneous oxidation of tetrahydrobiopterin in aqueous solution: kinetic characteristics and potentiation by catalase of ascorbate action.

  The international journal of biochemistry & cell biology 20040701

• Tetrahydrobiopterin improves endothelial dysfunction and vascular oxidative stress in microvessels of intrauterine undernourished rats.

  The Journal of physiology 20040701

• Synergistic interaction between enalapril, L-arginine and tetrahydrobiopterin in smooth muscle cell apoptosis and aortic remodeling induction in SHR.

  British journal of pharmacology 20040701

• Analysis of the kinetics of CO binding to neuronal nitric oxide synthase by flash photolysis: dual effects of substrates, inhibitors, and tetrahydrobiopterin.

  Journal of inorganic biochemistry 20040701

• CO exchange of the oxyferrous complexes of endothelial nitric-oxide synthase oxygenase domain in the presence of 4-amino-tetrahydrobiopterin.

  Journal of inorganic biochemistry 20040701

• Congenic mapping and genotyping of the tetrahydrobiopterin-deficient hph-1 mouse.

  Molecular genetics and metabolism 20040701

• Homocysteine induces oxidative stress by uncoupling of NO synthase activity through reduction of tetrahydrobiopterin.

  Free radical biology & medicine 20040615

• Dysfunction of endothelial nitric oxide synthase and atherosclerosis.

  Arteriosclerosis, thrombosis, and vascular biology 20040601

• The metabolic and molecular bases of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency.

  Molecular genetics and metabolism 20040601

• Changes in the concentrations of tetrahydrobiopterin, the cofactor of tyrosine hydroxylase, in blood under physical stress and in depression.

  Annals of the New York Academy of Sciences 20040601

• Reduced NOS3 phosphorylation mediates reduced NO/cGMP signaling in mesenteric arteries of deoxycorticosterone acetate-salt hypertensive rats.

  Hypertension (Dallas, Tex. : 1979) 20040501

• Nitrotyrosine as a marker for peroxynitrite-induced neurotoxicity: the beginning or the end of the end of dopamine neurons?

  Journal of neurochemistry 20040501

• 1-Methyl-4-phenylpyridinium-induced apoptosis in cerebellar granule neurons is mediated by transferrin receptor iron-dependent depletion of tetrahydrobiopterin and neuronal nitric-oxide synthase-derived superoxide.

  The Journal of biological chemistry 20040430

• Loss of striatal dopaminergic fibers after intraventricular injection of tetrahydrobiopterin in rat brain.

  Neuroscience letters 20040408

• Inhibition of nuclear factor kappa B activation and inducible nitric oxide synthase transcription by prolonged exposure to high glucose in the human keratinocyte cell line HaCaT.

  The British journal of dermatology 20040401

• Role of tetrahydrobiopterin in adrenocorticotropic hormone-induced hypertension in the rat.

  Clinical and experimental hypertension (New York, N.Y. : 1993) 20040401

• [Tetrahydrobiopterin and endothelial dysfunction].

  Sheng li ke xue jin zhan [Progress in physiology] 20040401

• Endothelial dysfunction in heart failure: mechanisms and therapeutic approaches.

  Current vascular pharmacology 20040401

• Regulation of endothelial nitric oxide synthase by tetrahydrobiopterin in vascular disease.

  Arteriosclerosis, thrombosis, and vascular biology 20040301

• Long-term treatment and diagnosis of tetrahydrobiopterin-responsive hyperphenylalaninemia with a mutant phenylalanine hydroxylase gene.

  Pediatric research 20040301

• Increased endothelial tetrahydrobiopterin synthesis by targeted transgenic GTP-cyclohydrolase I overexpression reduces endothelial dysfunction and atherosclerosis in ApoE-knockout mice.

  Arteriosclerosis, thrombosis, and vascular biology 20040301

• Fluvoxamine, a selective serotonin reuptake inhibitor, suppresses tetrahydrobiopterin in the mouse hippocampus.

  Neuropharmacology 20040301

• JNK activation by tetrahydrobiopterin: implication for Parkinson's disease.

  Journal of neuroscience research 20040301

• Tetrahydrobiopterin: mediator of endothelial protection.

  Arteriosclerosis, thrombosis, and vascular biology 20040301

• [Involvement of homocysteine in the pathogenesis of hypertension and hypertensive target-organ damage].

  Nihon rinsho. Japanese journal of clinical medicine 20040301

• [NO and hypertension].

  Nihon rinsho. Japanese journal of clinical medicine 20040301

• Tetrahydrobiopterin biosynthesis in white and brown adipose tissues is enhanced following intraperitoneal administration of bacterial lipopolysaccharide.

  Biochimica et biophysica acta 20040224

• Indirect oxidation of 6-tetrahydrobiopterin by tyrosinase.

  Biochemical and biophysical research communications 20040220

• Dynamic regulation of the inducible nitric-oxide synthase by NO: comparison with the endothelial isoform.

  The Journal of biological chemistry 20040206

• In vivo expression and function of recombinant GTPCH I in the rabbit carotid artery.

  American journal of physiology. Heart and circulatory physiology 20040201

• Long-term follow-up of a patient with mild tetrahydrobiopterin-responsive phenylketonuria.

  Molecular genetics and metabolism 20040201

• More transgenic mouse models of dopamine deficiency.

  Annals of neurology 20040201

• Perturbed 6-tetrahydrobiopterin recycling via decreased dihydropteridine reductase in vitiligo: more evidence for H2O2 stress.

  The Journal of investigative dermatology 20040201

• Nuclear localization of tetrahydrobiopterin biosynthetic enzymes.

  Biochimica et biophysica acta 20040105

• Activity of synthetic enzymes of tetrahydrobiopterin in the human placenta.

  International journal of molecular medicine 20040101

• Glucocorticoids decrease GTP cyclohydrolase and tetrahydrobiopterin-dependent vasorelaxation through glucocorticoid receptors.

  Journal of cardiovascular pharmacology 20040101

• Sepiapterin decreases vasorelaxation in nitric oxide synthase inhibition-induced hypertension.

  Journal of cardiovascular pharmacology 20040101

• GTP cyclohydrolase I utilizes metal-free GTP as its substrate.

  European journal of biochemistry 20040101

• New approaches to treat PKU: how far are we?

  Molecular genetics and metabolism 20040101

• Plasma tetrahydrobiopterin and its pharmacokinetic following oral administration.

  Molecular genetics and metabolism 20040101

• The fate of intravenously administered tetrahydrobiopterin and its implications for heterologous gene therapy of phenylketonuria.

  Molecular genetics and metabolism 20040101

• Tetrahydrobiopterin deficiency and dopamine loss in a genetic mouse model of Lesch-Nyhan disease.

  Journal of inherited metabolic disease 20040101

• Efficiency of long-term tetrahydrobiopterin monotherapy in phenylketonuria.

  Journal of inherited metabolic disease 20040101

• The two faces of endothelial nitric oxide synthase in the pathophysiology of atherosclerosis.

  Endothelium : journal of endothelial cell research 20040101

• Wild-type phenylalanine hydroxylase activity is enhanced by tetrahydrobiopterin supplementation in vivo: an implication for therapeutic basis of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency.

  Molecular genetics and metabolism 20040101

• Tetrahydrobiopterin responsiveness: results of the BH4 loading test in 31 Spanish PKU patients and correlation with their genotype.

  Molecular genetics and metabolism 20040101

• Regulation of tetrahydrobiopterin synthesis and bioavailability in endothelial cells.

  Cell biochemistry and biophysics 20040101

• Radical reactions of nitric oxide synthases.

  Biochemical Society symposium 20040101

• Tetrahydrobiopterin, a critical factor in the production and role of nitric oxide in mast cells.

  The Journal of biological chemistry 20031212

• Utilization of exogenous tetrahydrobiopterin in nitric oxide synthesis in human neuroblastoma cell line.

  Neuroscience letters 20031204

• Exogenous and intracellularly generated sphingosine 1-phosphate can regulate cellular processes by divergent pathways.

  Biochemical Society transactions 20031201

• Bacterial translocation up-regulates GTP-cyclohydrolase I in mesenteric vasculature of cirrhotic rats.

  Hepatology (Baltimore, Md.) 20031201

• Structural studies on phenylalanine hydroxylase and implications toward understanding and treating phenylketonuria.

  Pediatrics 20031201

• Potential role of tetrahydrobiopterin in the treatment of maternal phenylketonuria.

  Pediatrics 20031201

• Tetrahydrobiopterin and nitric oxide: mechanistic and pharmacological aspects.

  Experimental biology and medicine (Maywood, N.J.) 20031201

• [Involvement of tetrahydrobiopterin in local change of endothelium-dependent vasorelaxation in pulmonary hypertension].

  Rossiiskii fiziologicheskii zhurnal imeni I.M. Sechenova 20031201

• A tetrahydrobiopterin radical forms and then becomes reduced during Nomega-hydroxyarginine oxidation by nitric-oxide synthase.

  The Journal of biological chemistry 20031121

• Ability of tetrahydrobiopterin analogues to support catalysis by inducible nitric oxide synthase: formation of a pterin radical is required for enzyme activity.

  Biochemistry 20031118

• Critical role of interleukin-1beta for transcriptional regulation of endothelial 6-pyruvoyltetrahydropterin synthase.

  Arteriosclerosis, thrombosis, and vascular biology 20031101

• Expression of phenylalanine hydroxylase (PAH) in erythrogenic bone marrow does not correct hyperphenylalaninemia in Pah(enu2) mice.

  The journal of gene medicine 20031101

• cGMP inhibits GTP cyclohydrolase I activity and biosynthesis of tetrahydrobiopterin in human umbilical vein endothelial cells.

  Journal of pharmacological sciences 20031101

• 2.0A resolution crystal structures of the ternary complexes of human phenylalanine hydroxylase catalytic domain with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine or L-norleucine: substrate specificity and molecular motions related to substrate binding.

  Journal of molecular biology 20031031

• Ascorbate enhances iNOS activity by increasing tetrahydrobiopterin in RAW 264.7 cells.

  Free radical biology & medicine 20031015

• Role of nitric oxide, tetrahydrobiopterin and peroxynitrite in glucose toxicity-associated contractile dysfunction in ventricular myocytes.

  Diabetologia 20031001

• Tetrahydrobiopterin prevents nitration of tyrosine hydroxylase by peroxynitrite and nitrogen dioxide.

  Molecular pharmacology 20031001

• Chronic oral supplementation with sepiapterin prevents endothelial dysfunction and oxidative stress in small mesenteric arteries from diabetic (db/db) mice.

  British journal of pharmacology 20031001

• Genetic engineering of Escherichia coli for production of tetrahydrobiopterin.

  Metabolic engineering 20031001

• Role of ascorbate in the regulation of nitric oxide generation by polymorphonuclear leukocytes.

  Biochemical and biophysical research communications 20030912

• Gene transfer of human guanosine 5'-triphosphate cyclohydrolase I restores vascular tetrahydrobiopterin level and endothelial function in low renin hypertension.

  Circulation 20030909

• Mechanism of aromatic hydroxylation by an activated FeIV=O core in tetrahydrobiopterin-dependent hydroxylases.

  Chemistry (Weinheim an der Bergstrasse, Germany) 20030905

• Tetrahydrobiopterin-dependent preservation of nitric oxide-mediated endothelial function in diabetes by targeted transgenic GTP-cyclohydrolase I overexpression.

  The Journal of clinical investigation 20030901

• Immune function in children with classical phenylketonuria and tetrahydrobiopterin deficiencies.

  Indian pediatrics 20030901

• Tetrahydrobiopterin is synthesized from 6-pyruvoyl-tetrahydropterin by the human aldo-keto reductase AKR1 family members.

  Archives of biochemistry and biophysics 20030815

• Low tetrahydrobiopterin biosynthetic capacity of human monocytes is caused by exon skipping in 6-pyruvoyl tetrahydropterin synthase.

  The Biochemical journal 20030801

• Tetrahydrobiopterin restores endothelial dysfunction induced by an oral glucose challenge in healthy subjects.

  American journal of physiology. Heart and circulatory physiology 20030801

• Regulation of noradrenergic function by inflammatory cytokines and depolarization.

  Journal of neurochemistry 20030801

• Exogenous biopterins requirement for iNOS function in vascular smooth muscle cells.

  Journal of cardiovascular pharmacology 20030801

• Behavioral correction of Parkinsonian rats following the transplantation of immortalized fibroblasts genetically modified with TH and GCH genes.

  Parkinsonism & related disorders 20030801

• Kainic acid lesion-induced nigral neuronal death.

  Journal of chemical neuroanatomy 20030801

• Dwarfism and low insulin-like growth factor-1 due to dopamine depletion in Pts-/- mice rescued by feeding neurotransmitter precursors and H4-biopterin.

  The Journal of biological chemistry 20030725

• Mechanism of systemically injected interferon-alpha impeding monoamine biosynthesis in rats: role of nitric oxide as a signal crossing the blood-brain barrier.

  Brain research 20030718

• The autoxidation of tetrahydrobiopterin revisited. Proof of superoxide formation from reaction of tetrahydrobiopterin with molecular oxygen.

  The Journal of biological chemistry 20030704

• Dopamine-dependent cytotoxicity of tetrahydrobiopterin: a possible mechanism for selective neurodegeneration in Parkinson's disease.

  Journal of neurochemistry 20030701

• A potential mechanism for the impairment of nitric oxide formation caused by prolonged oral exposure to arsenate in rabbits.

  Free radical biology & medicine 20030701

• Degeneration of the nigrostriatal pathway and induction of motor deficit by tetrahydrobiopterin: an in vivo model relevant to Parkinson's disease.

  Neurobiology of disease 20030701

• A hypothesis on the biochemical mechanism of BH(4)-responsiveness in phenylalanine hydroxylase deficiency.

  Amino acids 20030701

• [Recent advances in cardiac protection].

  Nihon yakurigaku zasshi. Folia pharmacologica Japonica 20030701

• Physarum polycephalum expresses a dihydropteridine reductase with selectivity for pterin substrates with a 6-(1', 2'-dihydroxypropyl) substitution.

  Biological chemistry 20030701

• Characterization of Drosophila nitric oxide synthase: a biochemical study.

  Biochemical and biophysical research communications 20030627

• Interactions of peroxynitrite, tetrahydrobiopterin, ascorbic acid, and thiols: implications for uncoupling endothelial nitric-oxide synthase.

  The Journal of biological chemistry 20030620

• Overexpression of V-1 prevents nitric oxide-induced cell death: involvement of enhanced tetrahydrobiopterin biosynthesis.

  Journal of neuroscience research 20030615

• Probing cofactor specificity in phenylalanine hydroxylase by molecular dynamics simulations.

  Journal of biomolecular structure & dynamics 20030601

• Involvement of apoptosis and calcium mobilization in tetrahydrobiopterin-induced dopaminergic cell death.

  Experimental neurology 20030601

• Tetrahydrobiopterin radical enzymology.

  Chemical reviews 20030601

• [Neural and molecular mechanisms of fatigue and recovery from fatigue].

  Nihon shinkei seishin yakurigaku zasshi = Japanese journal of psychopharmacology 20030601

• Hydrogen peroxide stimulates tetrahydrobiopterin synthesis through the induction of GTP-cyclohydrolase I and increases nitric oxide synthase activity in vascular endothelial cells.

  Free radical biology & medicine 20030515

• Reactive oxygen species are involved in smoking-induced dysfunction of nitric oxide biosynthesis and upregulation of endothelial nitric oxide synthase: an in vitro demonstration in human coronary artery endothelial cells.

  Circulation 20030513

• Novel inhibitors of neuronal nitric oxide synthase.

  Experimental biology and medicine (Maywood, N.J.) 20030501

• Possible impact of tetrahydrobiopterin and sepiapterin on endothelial dysfunction.

  Arteriosclerosis, thrombosis, and vascular biology 20030501

• [Structure-function relationships of NO synthase: electron transfer reaction].

  Seikagaku. The Journal of Japanese Biochemical Society 20030501

• Tetrahydrobiopterin attenuates homocysteine induced endothelial dysfunction.

  Molecular and cellular biochemistry 20030501

• Tetrahydrobiopterin and mild phenylketonuria.

  The New England journal of medicine 20030424

• Tetrahydrobiopterin and mild phenylketonuria.

  The New England journal of medicine 20030424

• Oxidation of tetrahydrobiopterin leads to uncoupling of endothelial cell nitric oxide synthase in hypertension.

  The Journal of clinical investigation 20030415

• Two modes of binding of N-hydroxyguanidines to NO synthases: first evidence for the formation of iron-N-hydroxyguanidine complexes and key role of tetrahydrobiopterin in determining the binding mode.

  Biochemistry 20030408

• Tetrahydrobiopterin precursor sepiapterin provides protection against neurotoxicity of 1-methyl-4-phenylpyridinium in nigral slice cultures.

  Journal of neurochemistry 20030401

• Tetrahydrobiopterin sensitivity in German patients with mild phenylalanine hydroxylase deficiency.

  Human mutation 20030401

• Induction of LOX-1 and iNOS expressions by ischemia-reperfusion of rat kidney and the opposing effect of L-arginine.

  FASEB journal : official publication of the Federation of American Societies for Experimental Biology 20030401

• Nitric oxide synthase induction, cGMP elevation, and biopterin synthesis in vascular smooth muscle cells stimulated with interleukin-1beta in hypoxia.

  Journal of biochemistry 20030401

• [An eighteen-year study on phenylketonuria].

  Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae 20030401

• Role of human GTP cyclohydrolase I and its regulatory protein in tetrahydrobiopterin metabolism.

  Basic research in cardiology 20030301

• Modeled ligand-protein complexes elucidate the origin of substrate specificity and provide insight into catalytic mechanisms of phenylalanine hydroxylase and tyrosine hydroxylase.

  European journal of biochemistry 20030301

• Tetrahydrobiopterin-responsive hyperphenylalaninaemia due to homozygous mutations in the phenylalanine hydroxylase gene.

  European journal of pediatrics 20030301

• Stress and vascular responses: oxidative stress and endothelial dysfunction in the insulin-resistant state.

  Journal of pharmacological sciences 20030301

• BH4-sensitive hyperphenylalaninemia: new case and review of literature.

  Pediatric neurology 20030301

• New approaches to overcome tolerance to nitrates.

  Cardiovascular drugs and therapy 20030301

• Effect of proteasome inhibitor on cultured mesencephalic dopaminergic neurons.

  Brain research 20030227

• Improvement by repeated administration of 6R-tetrahydrobiopterin of 5,7-dihydroxytryptamine-induced abnormal behaviors in immature rats.

  Biochemical and biophysical research communications 20030227

• Structure of tetrahydrobiopterin tunes its electron transfer to the heme-dioxy intermediate in nitric oxide synthase.

  Biochemistry 20030225

• Diminished production of nitric oxide synthase cofactor tetrahydrobiopterin by rosiglitazone in adipocytes.

  Biochemical pharmacology 20030215

• Increased nitric oxide production and protection from malaria.

  Lancet (London, England) 20030215

• Impairment of nitric oxide synthase-dependent dilatation of cerebral arterioles during infusion of nicotine.

  American journal of physiology. Heart and circulatory physiology 20030201

• HMG-CoA reductase inhibitor increases GTP cyclohydrolase I mRNA and tetrahydrobiopterin in vascular endothelial cells.

  Arteriosclerosis, thrombosis, and vascular biology 20030201

• Tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency, state of the art.

  Molecular genetics and metabolism 20030201

• Role of tetrahydrobiopterin on ischemia-reperfusion injury in isolated perfused rat hearts.

  The Journal of cardiovascular surgery 20030201

• The role of tetrahydrobiopterin in superoxide generation from eNOS: enzymology and physiological implications.

  Free radical research 20030201

• Long-term vitamin C treatment increases vascular tetrahydrobiopterin levels and nitric oxide synthase activity.

  Circulation research 20030110

• Specific potentiation of endothelium-dependent contractions in SHR by tetrahydrobiopterin.

  Hypertension (Dallas, Tex. : 1979) 20030101

• A diffusible substance(s) mediates endothelium-dependent contractions in the aorta of SHR.

  Hypertension (Dallas, Tex. : 1979) 20030101

• Peripheral administration of lipopolysaccharide enhances the expression of guanosine triphosphate cyclohydrolase I mRNA in murine locus coeruleus.

  Neuroscience 20030101

• Tetrahydrobiopterin control in phenylketonuria.

  Genetics in medicine : official journal of the American College of Medical Genetics 20030101

• Lack of nitric oxide mediation of flow-dependent arteriolar dilation in type I diabetes is restored by sepiapterin.

  Journal of vascular research 20030101

• Effect of tetrahydrobiopterin on blood pressure in rats after subtotal nephrectomy.

  Nephron. Physiology 20030101

• Folic acid says NO to vascular diseases.

  Nutrition (Burbank, Los Angeles County, Calif.) 20030101

• Danger of high-protein dietary supplements to persons with hyperphenylalaninaemia.

  Journal of inherited metabolic disease 20030101

• Phenylketonuria detected by the neonatal screening program in Thailand.

  The Southeast Asian journal of tropical medicine and public health 20030101

• 6-pyruvoyl tetrahydropterin synthase deficiency: a case report.

  The Southeast Asian journal of tropical medicine and public health 20030101

• Tetrahydrobiopterin and dietary restriction in mild phenylketonuria.

  The New England journal of medicine 20021226

• Tetrahydrobiopterin as an alternative treatment for mild phenylketonuria.

  The New England journal of medicine 20021226

• Nitric oxide (NO) pretreatment increases cytokine-induced NO production in cultured rat hepatocytes by suppressing GTP cyclohydrolase I feedback inhibitory protein level and promoting inducible NO synthase dimerization.

  The Journal of biological chemistry 20021206

• High frequency of tetrahydrobiopterin-responsiveness among hyperphenylalaninemias: a study of 1,919 patients observed from 1988 to 2002.

  Molecular genetics and metabolism 20021201

• Mechanisms of superoxide production in human blood vessels: relationship to endothelial dysfunction, clinical and genetic risk factors.

  Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 20021201

• The antithyroid agent 6-n-propyl-2-thiouracil is a mechanism-based inactivator of the neuronal nitric oxide synthase isoform.

  Archives of biochemistry and biophysics 20021101

• Developmental regulation of neurotransmitter phenotype through tetrahydrobiopterin.

  The Journal of neuroscience : the official journal of the Society for Neuroscience 20021101

• Effect of 2,4-diamino-6-hydroxy-pyrimidine on postburn Staphylococcus aureus sepsis in rats.

  Critical care medicine 20021101

• The effect of insufficiency of tetrahydrobiopterin on endothelial function and vasoactivity.

  The Japanese journal of thoracic and cardiovascular surgery : official publication of the Japanese Association for Thoracic Surgery = Nihon Kyobu Geka Gakkai zasshi 20021101

• [Regulations and pathophysiological significance of the biosynthesis of tetrahydrobiopterin in human endothelial cells].

  Nihon yakurigaku zasshi. Folia pharmacologica Japonica 20021101

• [V-1, a catecholamine biosynthesis regulatory protein, enhances GTP cyclohydrolase I gene transcription in a cAMP-responsive element dependent manner].

  Nihon yakurigaku zasshi. Folia pharmacologica Japonica 20021101

• The role of tetrahydrobiopterin in the regulation of neuronal nitric-oxide synthase-generated superoxide.

  The Journal of biological chemistry 20021025

• Characterization of quinonoid-dihydropteridine reductase (QDPR) from the lower eukaryote Leishmania major.

  The Journal of biological chemistry 20021011

• Beneficial effect of tetrahydrobiopterin on ischemia-reperfusion injury in isolated perfused rat hearts.

  The Journal of thoracic and cardiovascular surgery 20021001

• Sepiapterin treatment in atherosclerosis.

  Arteriosclerosis, thrombosis, and vascular biology 20021001

• Altered tetrahydrobiopterin metabolism in atherosclerosis: implications for use of oxidized tetrahydrobiopterin analogues and thiol antioxidants.

  Arteriosclerosis, thrombosis, and vascular biology 20021001

• Why do nitric oxide synthases use tetrahydrobiopterin?

  Journal of inorganic biochemistry 20020920

• Stimulation of tetrahydrobiopterin synthesis by cyclosporin A in mouse brain microvascular endothelial cells.

  The international journal of biochemistry & cell biology 20020901

• GTP cyclohydrolase I gene transfer augments intracellular tetrahydrobiopterin in human endothelial cells: effects on nitric oxide synthase activity, protein levels and dimerisation.

  Cardiovascular research 20020901

• Tetrahydrobiopterin deficiency increases neuronal vulnerability to hypoxia.

  Journal of neurochemistry 20020901

• Glial cell line-derived neurotrophic factor up-regulates GTP-cyclohydrolase I activity and tetrahydrobiopterin levels in primary dopaminergic neurones.

  Journal of neurochemistry 20020901

• Possible involvement of tetrahydrobiopterin in the trophic effect of insulin-like growth factor-1 on rat pheochromocytoma-12 (PC12) cells.

  Neuroscience letters 20020809

• Overexpression of endothelial nitric oxide synthase accelerates atherosclerotic lesion formation in apoE-deficient mice.

  The Journal of clinical investigation 20020801

• A 4-amino analogue of tetrahydrobiopterin attenuates endotoxin-induced hemodynamic alterations and organ injury in rats.

  Shock (Augusta, Ga.) 20020801

• Oxidation of N-hydroxyguanidines by cytochromes P450 and NO-synthases and formation of nitric oxide.

  Drug metabolism reviews 20020801

• Tetrahydrobiopterin responsiveness in a large series of phenylketonuria patients.

  Journal of inherited metabolic disease 20020801

• Crystal structure of the ternary complex of the catalytic domain of human phenylalanine hydroxylase with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine, and its implications for the mechanism of catalysis and substrate activation.

  Journal of molecular biology 20020726

• Structural comparison of bacterial and human iron-dependent phenylalanine hydroxylases: similar fold, different stability and reaction rates.

  Journal of molecular biology 20020712

• 6R-Tetrahydrobiopterin induces dopamine synthesis in a human neuroblastoma cell line, LA-N-1. A cellular model of DOPA-responsive dystonia.

  Brain research 20020712

• Anti-inflammatory effects of tetrahydrobiopterin on early rejection in renal allografts: modulation of inducible nitric oxide synthase.

  FASEB journal : official publication of the Federation of American Societies for Experimental Biology 20020701

• Tetrahydrobiopterin monotherapy for phenylketonuria patients with common mild mutations.

  European journal of pediatrics 20020701

• Impaired vasodilation and nitric oxide synthase activity in glucocorticoid-induced hypertension.

  Biological research for nursing 20020701

• Binding of L-arginine and imidazole suggests heterogeneity of rat brain neuronal nitric oxide synthase.

  Biochemistry 20020618

• Interaction of 5-methyltetrahydrofolate and tetrahydrobiopterin on endothelial function.

  American journal of physiology. Heart and circulatory physiology 20020601

• Novel cardioprotective effects of tetrahydrobiopterin after anoxia and reoxygenation: Identifying cellular targets for pharmacologic manipulation.

  The Journal of thoracic and cardiovascular surgery 20020601

• Enhanced expression of GTP cyclohydrolase I in V-1-overexpressing PC12D cells.

  Biochemical and biophysical research communications 20020510

• Glutathione depletion in nigrostriatal slice cultures: GABA loss, dopamine resistance and protection by the tetrahydrobiopterin precursor sepiapterin.

  Brain research 20020510

• Direct evidence for nitric oxide production by a nitric-oxide synthase-like protein from Bacillus subtilis.

  The Journal of biological chemistry 20020503

• Cellular basis of endothelial dysfunction in small mesenteric arteries from spontaneously diabetic (db/db -/-) mice: role of decreased tetrahydrobiopterin bioavailability.

  British journal of pharmacology 20020501

• Evaluation of basic performance and applicability of a newly developed in vivo nitric oxide sensor.

  Physiological measurement 20020501

• Involvement of nitric oxide in glucose toxicity on differentiated PC12 cells: prevention of glucose toxicity by tetrahydrobiopterin, a cofactor for nitric oxide synthase.

  Neuroscience research 20020501

• Interleukin-1beta-induced nitric oxide production and inhibition of insulin secretion in rat islets of langerhans is dependent upon the nitric oxide synthase cofactor tetrahidrobiopterin.

  Cytokine 20020421

• Involvement of sphingosine kinase in TNF-alpha-stimulated tetrahydrobiopterin biosynthesis in C6 glioma cells.

  The Journal of biological chemistry 20020412

• Oxidative stress in vitiligo: photo-oxidation of pterins produces H(2)O(2) and pterin-6-carboxylic acid.

  Biochemical and biophysical research communications 20020412

• Mechanisms of increased vascular superoxide production in human diabetes mellitus: role of NAD(P)H oxidase and endothelial nitric oxide synthase.

  Circulation 20020409

• Tetrahydrobiopterin enhances forearm vascular response to acetylcholine in both normotensive and hypertensive individuals.

  American journal of hypertension 20020401

• Biopterin analogues: novel nitric oxide synthase inhibitors with immunosuppressive action.

  Current drug metabolism 20020401

• Disorders of tetrahydrobiopterin metabolism and their treatment.

  Current drug metabolism 20020401

• Tetrahydrobiopterin in nitric oxide synthesis: a novel biological role for pteridines.

  Current drug metabolism 20020401

• Tetrahydrobiopterin biosynthesis, utilization and pharmacological effects.

  Current drug metabolism 20020401

• Pteridine derivatives as modulators of oxidative stress.

  Current drug metabolism 20020401

• cAMP inhibits cytokine-induced biosynthesis of tetrahydrobiopterin in human umbilical vein endothelial cells.

  Life sciences 20020322

• The ratio between tetrahydrobiopterin and oxidized tetrahydrobiopterin analogues controls superoxide release from endothelial nitric oxide synthase: an EPR spin trapping study.

  The Biochemical journal 20020315

• Oxidation of the zinc-thiolate complex and uncoupling of endothelial nitric oxide synthase by peroxynitrite.

  The Journal of clinical investigation 20020315

• Reactions catalyzed by the heme domain of inducible nitric oxide synthase: evidence for the involvement of tetrahydrobiopterin in electron transfer.

  Biochemistry 20020312

• Nitric oxide (NO) traffic in endothelial NO synthase. Evidence for a new NO binding site dependent on tetrahydrobiopterin?

  The Journal of biological chemistry 20020301

• Evaluation of 6-year application of the enzymatic colorimetric phenylalanine assay in the setting of neonatal screening for phenylketonuria.

  Clinica chimica acta; international journal of clinical chemistry 20020301

• Tetrahydrobiopterin restores endothelial function of coronary arteries in patients with hypercholesterolaemia.

  Heart (British Cardiac Society) 20020301

• Glucose deprivation decreases nitric oxide production via NADPH depletion in immunostimulated rat primary astrocytes.

  Glia 20020301

• Tetrahydrobiopterin improves impaired endothelium-dependent forearm vasodilation in patients with heart failure.

  Journal of cardiovascular pharmacology 20020301

• Chemical stabilization of tetrahydrobiopterin by L-ascorbic acid: contribution to placental endothelial nitric oxide synthase activity.

  Molecular human reproduction 20020301

• Evidence for a role of nitric oxide in the mediation of antiproliferative UVA effects in keratinocytes.

  Pharmacological research 20020301

• Mental illness in mild PKU responds to biopterin.

  Molecular genetics and metabolism 20020301

• Mutations of GCH1 in Dopa-responsive dystonia.

  Journal of neural transmission (Vienna, Austria : 1996) 20020301

• Expression and activity of nitric oxide synthase isoforms in rat brain during the development of experimental allergic encephalomyelitis.

  Brain research. Molecular brain research 20020227

• [Role of tetrahydrobiopterin in the regulation of activity of human placental nitric oxide synthase in normal and pre-eclamptic pregnancies].

  Orvosi hetilap 20020224

• Oxidation of tetrahydrobiopterin by biological radicals and scavenging of the trihydrobiopterin radical by ascorbate.

  Free radical biology & medicine 20020201

• Tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency: possible regulation of gene expression in a patient with the homozygous L48S mutation.

  Molecular genetics and metabolism 20020201

• Screening for tetrahydrobiopterin deficiency among hyperphenylalaninemia patients in Southern China.

  Chinese medical journal 20020201

• Pathogenic theories and intrathecal analysis of the sporadic form of Alzheimer's disease.

  Progress in neurobiology 20020201

• Tetrahydrobiopterin responsiveness in phenylketonuria. Two new cases and a review of molecular genetic findings.

  Journal of inherited metabolic disease 20020201

• Nitrocatechols versus nitrocatecholamines as novel competitive inhibitors of neuronal nitric oxide synthase: lack of the aminoethyl side chain determines loss of tetrahydrobiopterin-antagonizing properties.

  Bioorganic & medicinal chemistry letters 20020107

• Citrulline is not the major product using the standard 'NOS activity' assay on renal cortical homogenates.

  American journal of physiology. Regulatory, integrative and comparative physiology 20020101

• Decreased plasma tetrahydrobiopterin in pregnant women is caused by impaired 6-pyruvoyl tetrahydropterin synthase activity.

  International journal of molecular medicine 20020101

• Folates and cardiovascular disease.

  Arteriosclerosis, thrombosis, and vascular biology 20020101

• Inhibition of N-methyl-D-aspartic acid-nitric oxide synthase in rat hippocampal slices by ethanol. Evidence for the involvement of tetrahydrobiopterin but not lipid peroxidation.

  Journal of biomedical science 20020101

• Reduced nitric oxide metabolites in CSF of patients with tetrahydrobiopterin deficiency.

  Journal of neurochemistry 20020101

• Tetrahydrobiopterin improves vascular endothelial function in ovariectomized rats.

  Journal of biomedical science 20020101

• [Screening of tetrahydrobiopterin deficiency among hyperphenylalaninemic patients].

  Annales de biologie clinique 20020101

• Plasma biopterin levels and depressive state in pregnancy and the early puerperal period.

  Neuropsychobiology 20020101

• Tetrahydrobiopterin improves coronary endothelial function, but does not prevent coronary spasm in patients with vasospastic angina.

  Circulation journal : official journal of the Japanese Circulation Society 20020101

• Cranial MR spectroscopy of tetrahydrobiopterin deficiency.

  AJNR. American journal of neuroradiology 20020101

• Redox role for tetrahydrobiopterin in nitric oxide synthase catalysis: low-temperature optical absorption spectral detection.

  Methods in enzymology 20020101

• Regulation of endothelial cell proliferation by nitric oxide.

  Methods in enzymology 20020101

• [Novel PKU treatment methods].

  Medycyna wieku rozwojowego 20020101

• [Atypical phenylketonuria treatment effectiveness].

  Medycyna wieku rozwojowego 20020101

• Structural basis for pterin antagonism in nitric-oxide synthase. Development of novel 4-oxo-pteridine antagonists of (6R)-5,6,7,8-tetrahydrobiopterin.

  The Journal of biological chemistry 20011228

• Impaired endothelial regulation of ventricular relaxation in cardiac hypertrophy: role of reactive oxygen species and NADPH oxidase.

  Circulation 20011211

• Tetrahydrobiopterin attenuates cholesterol induced coronary hyperreactivity to endothelin.

  Heart (British Cardiac Society) 20011201

• Mechanisms of synergistic cytokine-induced nitric oxide production in human alveolar epithelial cells.

  Nitric oxide : biology and chemistry 20011201

• Coronary endothelial dysfunction in the insulin-resistant state is linked to abnormal pteridine metabolism and vascular oxidative stress.

  Journal of the American College of Cardiology 20011201

• [Perspectives on tetrahydrobiopterin research].

  Nihon yakurigaku zasshi. Folia pharmacologica Japonica 20011201

• Oxidation of tetrahydrobiopterin by peroxynitrite or oxoferryl species occurs by a radical pathway.

  Free radical research 20011201

• Treatment and outcome of Taiwanese patients with 6-pyruvoyltetrahydropterin synthase gene mutations.

  Journal of inherited metabolic disease 20011201

• Involvement of tetrahydrobiopterin in trophic effect of erythropoietin on PC12 cells.

  Biochemical and biophysical research communications 20011130

• High resolution crystal structures of the catalytic domain of human phenylalanine hydroxylase in its catalytically active Fe(II) form and binary complex with tetrahydrobiopterin.

  Journal of molecular biology 20011123

• Crystal structure of nitric oxide synthase bound to nitro indazole reveals a novel inactivation mechanism.

  Biochemistry 20011113

• Intraplatelet tetrahydrobiopterin plays an important role in regulating canine coronary arterial thrombosis by modulating intraplatelet nitric oxide and superoxide generation.

  Circulation 20011113

• Catecholamines and serotonin are differently regulated by tetrahydrobiopterin. A study from 6-pyruvoyltetrahydropterin synthase knockout mice.

  The Journal of biological chemistry 20011102

• Tetrahydrobiopterin, a cofactor for NOS, improves endothelial dysfunction during chronic alcohol consumption.

  American journal of physiology. Heart and circulatory physiology 20011101

• Tetrahydrobiopterin-deficient hyperphenylalaninemia in the Chinese.

  Clinica chimica acta; international journal of clinical chemistry 20011101

• Characterization of GTP cyclohydrolase I gene expression in the human neuroblastoma SKN-BE(2)M17: enhanced transcription in response to cAMP is conferred by the proximal promoter.

  Journal of neurochemistry 20011101

• Supplemention with tetrahydrobiopterin suppresses the development of hypertension in spontaneously hypertensive rats.

  Hypertension (Dallas, Tex. : 1979) 20011101

• Current strategies and perspectives for correcting endothelial dysfunction in atherosclerosis.

  Journal of cardiovascular pharmacology 20011101

• A conserved tryptophan in nitric oxide synthase regulates heme-dioxy reduction by tetrahydrobiopterin.

  Biochemistry 20011030

• Structures of tetrahydrobiopterin binding-site mutants of inducible nitric oxide synthase oxygenase dimer and implicated roles of Trp457.

  Biochemistry 20011030

• Reaction of tetrahydrobiopterin with superoxide: EPR-kinetic analysis and characterization of the pteridine radical.

  Free radical biology & medicine 20011015

• Tetrahydrobiopterin scavenges superoxide in dopaminergic neurons.

  The Journal of biological chemistry 20010914

• Treatable neurotransmitter deficiency in mild phenylketonuria.

  Neurology 20010911

• Survival and graft function in a large animal lung transplant model after 30 h preservation and substitution of the nitric oxide pathway.

  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 20010901

• Vascular endothelial dysfunction: does tetrahydrobiopterin play a role?

  American journal of physiology. Heart and circulatory physiology 20010901

• Carboxy-PTIO increases the tetrahydrobiopterin level in mouse brain microvascular endothelial cells.

  Japanese journal of pharmacology 20010901

• Molecular cloning and disruption of a novel gene encoding UDP-glucose: tetrahydrobiopterin alpha-glucosyltransferase in the cyanobacterium Synechococcus sp. PCC 7942.

  FEBS letters 20010803

• Constitutive expression of ectopic c-Myc delays glucocorticoid-evoked apoptosis of human leukemic CEM-C7 cells.

  Oncogene 20010802

• Mutations in the sepiapterin reductase gene cause a novel tetrahydrobiopterin-dependent monoamine-neurotransmitter deficiency without hyperphenylalaninemia.

  American journal of human genetics 20010801

• Tetrahydrobiopterin levels regulate endothelial cell proliferation.

  American journal of physiology. Heart and circulatory physiology 20010801

• Effects of tetrahydrobiopterin on ataxia in Machado-Joseph disease may be based upon the theory of 'cerebellar long-term depression'.

  Medical hypotheses 20010801

• X-ray absorption spectroscopic analysis of the high-spin ferriheme site in substrate-bound neuronal nitric-oxide synthase.

  Journal of biochemistry 20010801

• Tetrahydrobiopterin improves endothelial dysfunction in coronary microcirculation in patients without epicardial coronary artery disease.

  Journal of the American College of Cardiology 20010801

• Determination of tetrahydrobiopterin in murine locus coeruleus by HPLC with fluorescence detection.

  Brain research. Brain research protocols 20010801

• [Orphan drugs and metabolic disorders].

  Revista de neurologia 20010801

• Use of surface plasmon resonance for real-time measurements of the global conformational transition in human phenylalanine hydroxylase in response to substrate binding and catalytic activation.

  Analytical biochemistry 20010715

• Tetrahydrobiopterin reverses the inhibition of nitric oxide by high glucose in cultured murine mesangial cells.

  American journal of physiology. Renal physiology 20010701

• Folate improves endothelial function in coronary artery disease: an effect mediated by reduction of intracellular superoxide?

  Arteriosclerosis, thrombosis, and vascular biology 20010701

• Role of nitric oxide synthase in the light-induced development of sporangiophores in Phycomyces blakesleeanus.

  Plant physiology 20010701

• Homomeric and heteromeric interactions between wild-type and mutant phenylalanine hydroxylase subunits: evaluation of two-hybrid approaches for functional analysis of mutations causing hyperphenylalaninemia.

  Molecular genetics and metabolism 20010701

• Tetrahydrobiopterin: a critical cofactor for eNOS and a strategy in the treatment of endothelial dysfunction?

  American journal of physiology. Heart and circulatory physiology 20010601

• Hypoxia impairs endothelium-dependent relaxation in organ cultured pulmonary artery.

  European journal of pharmacology 20010601

• Tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency in Dutch neonates.

  Journal of inherited metabolic disease 20010601

• Pterin interactions with distinct reductase activities of NO synthase.

  The Biochemical journal 20010515

• Inhibition of neuronal nitric oxide synthase by 6-nitrocatecholamines, putative reaction products of nitric oxide with catecholamines under oxidative stress conditions.

  The Biochemical journal 20010515

• Tetrahydrobiopterin augments arginine transport in rat cardiac myocytes through modulation of CAT-2 mRNA.

  The Journal of laboratory and clinical medicine 20010501

• NO or H(2)O(2) for endothelium-dependent vasorelaxation: Tetrahydrobiopterin makes the difference.

  Arteriosclerosis, thrombosis, and vascular biology 20010501

• Successful treatment of phenylketonuria with tetrahydrobiopterin.

  European journal of pediatrics 20010501

• Hypochlorite-modified low density lipoprotein inhibits nitric oxide synthesis in endothelial cells via an intracellular dislocalization of endothelial nitric-oxide synthase.

  The Journal of biological chemistry 20010427

• Important role of tetrahydrobiopterin in no complex formation and interdomain electron transfer in neuronal nitric-oxide synthase.

  Biochemical and biophysical research communications 20010420

• Regulation of differentiation to the infective stage of the protozoan parasite Leishmania major by tetrahydrobiopterin.

  Science (New York, N.Y.) 20010413

• Thiouracil antithyroid drugs as a new class of neuronal nitric oxide synthase inhibitors.

  Biochemical and biophysical research communications 20010406

• Ligand binding to the inhibitory and stimulatory GTP cyclohydrolase I/GTP cyclohydrolase I feedback regulatory protein complexes.

  Protein science : a publication of the Protein Society 20010401

• Electrochemistry of pterin cofactors and inhibitors of nitric oxide synthase.

  Nitric oxide : biology and chemistry 20010401

• [Studies on neonatal screening, clinical and gene analysis for tetrahydrobiopterin deficiency in Southern Chinese].

  Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 20010401

• Reactive oxygen species mediate endothelium-dependent relaxations in tetrahydrobiopterin-deficient mice.

  Arteriosclerosis, thrombosis, and vascular biology 20010401

• Tetrahydrobiopterin reverses the impairment of acetylcholine-induced vasodilatation in diabetic ocular microvasculature.

  Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics 20010401

• A structural hypothesis for BH4 responsiveness in patients with mild forms of hyperphenylalaninaemia and phenylketonuria.

  Journal of inherited metabolic disease 20010401

• Circulating tetrahydrobiopterin concentrations in patients with septic shock.

  British journal of anaesthesia 20010401

• Tetrahydrobiopterin improves endothelium-dependent vasodilation in nitroglycerin-tolerant rats.

  European journal of pharmacology 20010330

• Tetrahydrobiopterin enhances apoptotic PC12 cell death following withdrawal of trophic support.

  The Journal of biological chemistry 20010323

• Endothelial regulation of vasomotion in apoE-deficient mice: implications for interactions between peroxynitrite and tetrahydrobiopterin.

  Circulation 20010306

• The cytotoxic effects of diesel exhaust particles on human pulmonary artery endothelial cells in vitro: role of active oxygen species.

  Free radical biology & medicine 20010301

• Diagnosis of dopa-responsive dystonia and other tetrahydrobiopterin disorders by the study of biopterin metabolism in fibroblasts.

  Clinical chemistry 20010301

• Effect of vitamin C on the availability of tetrahydrobiopterin in human endothelial cells.

  Journal of cardiovascular pharmacology 20010301

• Differential nitric oxide synthase activity, cofactor availability and cGMP accumulation in the central nervous system during anaesthesia.

  British journal of anaesthesia 20010301

• Tetrahydrobiopterin increases myocardial blood flow in healthy volunteers: a double-blind, placebo-controlled study.

  Swiss medical weekly 20010224

• Kinetics of CO and NO ligation with the Cys(331)-->Ala mutant of neuronal nitric-oxide synthase.

  The Journal of biological chemistry 20010216

• Expression of GTP cyclohydrolase I in murine locus ceruleus is enhanced by peripheral administration of lipopolysaccharide.

  Brain research 20010202

• Tetrahydrobiopterin attenuates modulation of platelet 12-lipoxygenase and cyclooxygenase activities by nitric oxide.

  Nitric oxide : biology and chemistry 20010201

• Paracrine neuroprotective effect of nitric oxide in the developing retina.

  Journal of neurochemistry 20010201

• The optical interconversion of the P-450 and P-420 forms of neuronal nitric oxide synthase: effects of sodium cholate, mercury chloride and urea.

  The international journal of biochemistry & cell biology 20010201

• Oxidations of N(omega)-hydroxyarginine analogues and various N-hydroxyguanidines by NO synthase II: key role of tetrahydrobiopterin in the reaction mechanism and substrate selectivity.

  Chemical research in toxicology 20010201

• Estrogenic activity of phenolic additives determined by an in vitro yeast bioassay.

  Environmental health perspectives 20010201

• Essential role of the N-terminal autoregulatory sequence in the regulation of phenylalanine hydroxylase.

  FEBS letters 20010119

• Rapid kinetic studies link tetrahydrobiopterin radical formation to heme-dioxy reduction and arginine hydroxylation in inducible nitric-oxide synthase.

  The Journal of biological chemistry 20010105

• L-ascorbic acid potentiates endothelial nitric oxide synthesis via a chemical stabilization of tetrahydrobiopterin.

  The Journal of biological chemistry 20010105

• Reconstitution of pterin-free inducible nitric-oxide synthase.

  The Journal of biological chemistry 20010105

• Stimulation of tetrahydrobiopterin synthesis induced by insulin: possible involvement of phosphatidylinositol 3-kinase.

  The international journal of biochemistry & cell biology 20010101

• Epidermal H(2)O(2) accumulation alters tetrahydrobiopterin (6BH4) recycling in vitiligo: identification of a general mechanism in regulation of all 6BH4-dependent processes?

  The Journal of investigative dermatology 20010101

• CSF dopamine, serotonin, and biopterin metabolites in patients with restless legs syndrome.

  Movement disorders : official journal of the Movement Disorder Society 20010101

• Prolonged survival of murine cardiac allografts by treatment with the 4-amino analog of tetrahydrobiopterin.

  Transplantation proceedings 20010101

• Glucocorticoids inhibit tetrahydrobiopterin-dependent endothelial function.

  Experimental biology and medicine (Maywood, N.J.) 20010101

• Parallel induction of nitric oxide and tetrahydrobiopterin synthesis in alveolar macrophages.

  Respiration; international review of thoracic diseases 20010101

• Identification of three novel 6-pyruvoyl-tetrahydropterin synthase gene mutations (226C>T, IVS3+1G>A, 116-119delTGTT) in Chinese hyperphenylalaninemia caused by tetrahydrobiopterin synthesis deficiency.

  Human mutation 20010101

• Tetrahydrobiopterin deficiencies without hyperphenylalaninemia: diagnosis and genetics of dopa-responsive dystonia and sepiapterin reductase deficiency.

  Molecular genetics and metabolism 20010101

• Tetrahydrobiopterin biosynthesis, regeneration and functions.

  The Biochemical journal 20000401

• Abnormal biopterin metabolism is a major cause of impaired endothelium-dependent relaxation through nitric oxide/O2- imbalance in insulin-resistant rat aorta.

  Diabetes 19991201

• Nitric oxide-induced inhibition of lung endothelial cell nitric oxide synthase via interaction with allosteric thiols: role of thioredoxin in regulation of catalytic activity.

  American journal of respiratory cell and molecular biology 19960901

• Guanosine triphosphate cyclohydrolase I regulates nitric oxide synthesis in renal proximal tubules.

  Journal of the American Society of Nephrology : JASN 19950201

• Tetrahydrobiopterin cofactor biosynthesis: GTP cyclohydrolase I mRNA expression in rat brain and superior cervical ganglia.

  Journal of neurochemistry 19930901

• Substitutive therapy in a case of methotrexate neurotoxicity.

  European journal of cancer (Oxford, England : 1990) 19920101

• [Early neurotoxicity of high-dose of methotrexate and tetrahydrobiopterin deficiency].

  Archives francaises de pediatrie 19911201

• The biosynthesis of tetrahydrobiopterin in rat brain. Purification and characterization of 6-pyruvoyl tetrahydropterin (2'-oxo)reductase.

  The Journal of biological chemistry 19890515