Keywords: BH4، تتراهیدروبیوپترین; Phenylketonuria; PKU diet; Cardiovascular risk; Nutrition; Obesity; Lipid profileADMA, asymmetric dimethylarginine; BH4, tetrahydrobiopterin; BMI, body mass index; CVD, cardiovascular disease; HDL-C, high-density lipoprotein cholesterol; HPA, hyperphenyla
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Keywords: BH4، تتراهیدروبیوپترین; Hypoxia; Oxidative stress; Animal models; Pharmacological strategiesBH2, dehydrobiopterin; BH4, tetrahydrobiopterin; CAO, cerebral artery occlusion; CAT, catalase; CKD, chronic kidney disease; COX, cycloxygenase; CuZnSOD, superoxide dismutase; eNOS, endot
Keywords: BH4، تتراهیدروبیوپترین; Isolated endothelial cells; Chronic exercise; RatCellules endothéliales isolées; Exercice chronique; RatAch, Acetylcholine; BH4, Tetrahydrobiopterin; ED, Endothelial Dysfunction; EDHF, Endothelium-Derived Hyperpolarizing Factor; eNOS, NO Synthase Endothel
Keywords: BH4، تتراهیدروبیوپترین; HPA, hyperphenylalaninemia; PAH, phenylalanine hydroxylase; PKU, phenylketonuria; BH4, tetrahydrobiopterin; GTPCH, guanosine 5′-triphosphate cyclohydrolase I; PTPS, 6-pyruvoyl-tetrahydropterin synthase; PCD, pterin-4-carbinolamine dehydratase; DHPR, dihyd
Keywords: BH4، تتراهیدروبیوپترین; Ach, Acetylcholine; ADMA, Asymmetrical dimethylarginine; BH4, Tetrahydrobiopterin; CAD, Coronary artery disease; CBF, Coronary blood flow; CR, Cardiac rehabilitation; CRF, Cardiorespiratory fitness; CSM, Coronary smooth muscle cells; CV, Cardiovascular; C
Keywords: BH4، تتراهیدروبیوپترین; AA, African Americans; ACh, acetylcholine; ACE, angiotensin converting enzyme; BH4, tetrahydrobiopterin; BP, blood pressure; cGMP, cyclic guanosin monophosphate; CHD, coronary heart disease; CRP, C-reactive protein; CV, cardiovascular; CVD, cardiovascular
Keywords: BH4، تتراهیدروبیوپترین; BH4, tetrahydrobiopterin; CRP, C-reactive protein; COX, cyclo-oxygenase; ER, endoplasmic reticulum; ERO1, endoplasmic reticulum oxidoreductin 1; EV, extracellular vesicles; FX1, factor XI; GPI, glycoprotein 1; GPX, glutathione peroxidase; GRX, glutaredoxi
Keywords: BH4، تتراهیدروبیوپترین; AKT, RAC-alpha serine/threonine-protein kinase; AP-1, activator protein-1; ATF6, activating transcription factor 6; BH4, tetrahydrobiopterin; BigET1, big endothelin-1; CDC42, cell division control protein 42 homolog; DAPK, death associated protein kinase;
Keywords: BH4، تتراهیدروبیوپترین; Atherosclerosis; Cardiovascular disease; Endothelial function; Therapeutic approachesACEIs, angiotensin-converting enzyme inhibitors; ADMA, asymmetrical dimethyl arginine; ARBs, angiotensin-receptor antagonists; BH4, tetrahydrobiopterin; CAD, coronary art
A novel common large genomic deletion and two new missense mutations identified in the Romanian phenylketonuria population
Keywords: BH4، تتراهیدروبیوپترین; PAH, phenylalanine hydroxylase; HPA, hyperphenylalaninemia; PKU, phenylketonuria; BH4, tetrahydrobiopterin; L-Phe, phenylalanine; MLPA, multiplex ligation-dependent probe amplification; NGS, next generation sequencingHyperphenylalaninemia; Phenylketonuria
Identification and molecular characterization of nitric oxide synthase (NOS) gene in the intertidal copepod Tigriopus japonicus
Keywords: BH4، تتراهیدروبیوپترین; iNOS, inducible nitric oxide synthase; NO, nitric oxide; LPS, lipopolysaccharide; NADPH, nicotinamide adenine dinucleotide phosphate; FAD, flavin adenine dinucleotide; BH4, tetrahydrobiopterin; CaM, calmodulin; CDD, Conserved Domain Database; GTR, general
Nitric Oxide and ERK mediates regulation of cellular processes by Ecdysterone
Keywords: BH4، تتراهیدروبیوپترین; NO, Nitric Oxide; NOS, Nitric Oxide Synthase; 3T3-L1, Mouse embryonic fibroblast cell line; MTT, 3-(4,5-dimethyl-2-thiazolyl)-2,5-diphenyl-H-2-tetrazolium bromide; BH4, Tetrahydrobiopterin; DAF FM diacetate, 4-amino-5-methylamino- 2′,7′ -difluorofluoresce
Chromatographic behavior of 12 polar pteridines in hydrophilic interaction chromatography using five different HILIC columns coupled with tandem mass spectrometry
Keywords: BH4، تتراهیدروبیوپترین; HILIC, hydrophilic interaction chromatography; LC–MS/MS, liquid chromatography–tandem mass spectrometry; Neo, neopterin; NH2, dihyroneopterin; Bio, biopterin; BH2, dihyrobiopterin; BH4, tetrahydrobiopterin; Xan, xanthopterin; Iso, isoxanthopterin; Pt, pte
2-Acetyl-7-hydroxy-6-methoxy-1-methyl-1,2,3,4,-tetrahydroisoquinoline exhibits anti-inflammatory properties and protects the nigral dopaminergic neurons
Keywords: BH4، تتراهیدروبیوپترین; AMTIQ, 2-acetyl-7-hydroxy-6-methoxy-1-methyl-1,2,3,4,-tetarhydroisoquinoline; BH4, tetrahydrobiopterin; CYP, cytochrome P450; DAergic, dopaminergic; ELISA, enzyme-linked immuno sorbent assay; GTPCH, GTP cyclohydrolase I, IL-1β, interleukin-1β; iNOS, induc
Antidepressant therapies inhibit inflammation and microglial M1-polarization
Keywords: BH4، تتراهیدروبیوپترین; 5HT, serotonin; BH4, tetrahydrobiopterin; CBP, CREB-binding protein; CREB, cAMP response element-binding protein; DA, dopamine; ECT, electroconvulsive shock therapy; GR, glucocorticoid-receptor; GSK3 β, glycogen synthase kinase-3β; HAT, histone acetyltran
Endothelial nitric oxide synthase: From biochemistry and gene structure to clinical implications of NOS3 polymorphisms
Keywords: BH4، تتراهیدروبیوپترین; 3′-UTR, 3′-mRNA untranslated region; 5′-UTR, 5′-mRNA untranslated region; ACE, angiotensin-converting enzyme; ARB, angiotensin II receptor blocker; BH4, tetrahydrobiopterin; cGMP, cyclic guanosine monophosphate; NOS3, endotelial nitric oxide synthase; FAD
The challenge of long-term tetrahydrobiopterin (BH4) therapy in phenylketonuria: Effects on metabolic control, nutritional habits and nutrient supply
Keywords: BH4، تتراهیدروبیوپترین; AAM, amino acid mixture; BH4, tetrahydrobiopterin; Phe, phenylalanine; PKU, phenylketonuria
Role of GTP-CHI links PAH and TH in melanin synthesis in silkworm, Bombyx mori
Keywords: BH4، تتراهیدروبیوپترین; BH4, tetrahydrobiopterin; GTP, guanosine triphosphate; H2NTP, 7,8-dihydroneopterin triphosphate; PTP, 6-pyruvoyl-5,6,7,8-tetrahydropterin; 4-OH-BH4, terin-4a-carbinolamine; q-BH2, quinonoid dihydrobiopterin; GTP-CH I, guanosine triphosphate cyclohydrolase
Perturbations in dopamine synthesis lead to discrete physiological effects and impact oxidative stress response in Drosophila
Keywords: BH4، تتراهیدروبیوپترین; BH4, tetrahydrobiopterin; Cat, catalase; Clk, clock; Cu/Zn SOD, copper–zinc superoxide dismutase; DA, dopamine; DD, constant darkness; FFT, Fast Fourier Transform; GSTO1, glutathione S-transferase Omega-1; GTPCH, guanosine triphosphate cyclohydrolase; HP,
The balance of powers: Redox regulation of fibrogenic pathways in kidney injury
Keywords: BH4، تتراهیدروبیوپترین; AGEs, advanced glycation end-products; ALE, advanced lipoxidation end-products; BH4, tetrahydrobiopterin; CML, Nε-(carboxymethyl)lysine; eNOS, endothelial nitric oxide synthase; ESRD, end stage renal disease; MS, mass spectrometry; HO•, hydroxyl radical;
Sample preparation and UHPLC-FD analysis of pteridines in human urine
Keywords: BH4، تتراهیدروبیوپترین; ACN, acetonitrile; BEH, ethylene bridged hybrid; BH2, dihydrobiopterin; BH4, tetrahydrobiopterin; BIO, biopterin; CREA, creatinine; DTT, dithiothreitol; EMA, European Medicines Agency; FD, fluorescence detection; HILIC, hydrophilic interaction liquid chro
Targeting NOS as a therapeutic approach for heart failure
Keywords: BH4، تتراهیدروبیوپترین; cGMP; S-nitrosylation; Contraction; Arrhythmia; Hypertrophy; Nitroso-redox balanceAP, action potential; β-AR, β-adrenergic; BH4, tetrahydrobiopterin; cGMP, cyclic GMP; DAD, delayed afterdepolarization; EAD, early afterdepolarization; ECC, excitation contr
Spectrum of mutations in Lebanese patients with phenylalanine hydroxylase deficiency
Keywords: BH4، تتراهیدروبیوپترین; AV, Arbitrary Value; BH4, Tetrahydrobiopterin; HR, Homozygosity rate; IVS, Intervening sequences; MHP, Mild Hyperphenylalaninemia; PAH, Phenylalanine hydroxylase; Phe, Phenylalanine; PKU, phenylketonuria; PRA, Predicted residual activityPhenylalanine; PKU
Alzheimer’s disease treated patients showed different patterns for oxidative stress and inflammation markers
Keywords: BH4، تتراهیدروبیوپترین; AD, Alzheimer’s diseases; ADMA, asymmetric dymethylarginine; AGEs, advanced glycation end-products; BH2, dihydrobiopterin; BH4, tetrahydrobiopterin; CRBNLs, protein carbonyls; CVD, cardiovascular disease; DDAHs, dimethylarginine dimethylaminohydrolases; D
Uric acid attenuates nitric oxide production by decreasing the interaction between endothelial nitric oxide synthase and calmodulin in human umbilical vein endothelial cells: A mechanism for uric acid-induced cardiovascular disease development
Keywords: BH4، تتراهیدروبیوپترین; NO, nitric oxide; eNOS, endothelial NO synthase; HUVEC, human umbilical vascular endothelial cell(s); BAEC, bovine aortic endothelial cell(s); CaM, calmodulin; BH4, tetrahydrobiopterin; CRP, C-reactive proteinUric acid; Nitric oxide; Endothelial nitric ox
Oxygen dependence of nitric oxide-mediated signaling
Keywords: BH4، تتراهیدروبیوپترین; BH4, tetrahydrobiopterin; cGMP, cyclic guanosine monophosphate; DETA/NO, (Z)-1-[N-(2-aminoethyl)–N-(2-ammonioethyl)amino]diazen-1-ium-1,2-diolate; eNOS, endothelial nitric oxide synthase; FAD, flavin adenine dinucleotide; FMN, flavin mononucleotide; iNOS,
Glu434 is an important amino acid residue for the activity, structure and stability of tyrosine hydroxylase of the silkworm, Bombyx mori
Keywords: BH4، تتراهیدروبیوپترین; l-DOPA, 3,4-dihydroxyphenylalanine; BH4, tetrahydrobiopterin; GdnHCl, guanidine hydrochlorideTyrosine hydroxylase; Silkworm; Bombyx mori; Aggregation; Folding intermediate; Structural stability
Guinea pig ascorbate status predicts tetrahydrobiopterin plasma concentration and oxidation ratio in vivo
Keywords: BH4، تتراهیدروبیوپترین; BH2, dihydrobiopterin; BH4, tetrahydrobiopterin; DHA, dehydroascorbic acid; DTE, dithioerythritol; ED, endothelial dysfunction; eNOS, endothelial nitric oxide synthase; HPLC, high performance liquid chromatography; MPA, meta-phosphoric acid; NOS, nitric o
Diabetes Mellitus-Associated Vascular Impairment : Novel Circulating Biomarkers and Therapeutic Approaches
Keywords: BH4، تتراهیدروبیوپترین; biomarkers; diabetes mellitus; endothelium; vascular functionADMA, asymmetric dimethylarginine; BH4, tetrahydrobiopterin; CAD, coronary artery disease; CRP, C-reactive protein; DM, diabetes mellitus; eNOS, endothelial nitric oxide synthase; EMP, endotheli
The impact of acute mental stress on vascular endothelial function: Evidence, mechanisms and importance
Keywords: BH4، تتراهیدروبیوپترین; ACh, acetylcholine; ACTH, adrenocorticotropic hormone; AUC, area under the curve; BH4, tetrahydrobiopterin; CRH, corticotropin-releasing hormone; DEX, dexamethasone; eNOS, endothelial nitric oxide synthase; ET-1, endothelin-1; ETA, endothelin A receptor;
Effects of 4-hydroxynonenal on vascular endothelial and smooth muscle cell redox signaling and function in health and disease
Keywords: BH4، تتراهیدروبیوپترین; Akt, Protein kinase B; AR, Aldose reductase; ARE, Antioxidant response element; AP-1, Activator protein-1; ATF6, Activating transcription factor 6; BH4, Tetrahydrobiopterin; BAEC, Bovine aortic endothelial cells; BLMVEC, Bovine lung microvascular vein end
Teaching the basics of redox biology to medical and graduate students: Oxidants, antioxidants and disease mechanisms
Keywords: BH4، تتراهیدروبیوپترین; 4-HNE, hydroxynonenol; 8-OHdG, 8-hydroxy-2-deoxyguanosine; ATP, adenosine triphosphate; BH4, tetrahydrobiopterin; CAT, catalase; CGD, chronic granulomatous disease; cGMP, cyclic GMP; CKD, chronic kidney disease; CO2, carbon dioxide; CO3–, carbonate radica
Carbon monoxide: Mechanisms of action and potential clinical implications
Keywords: BH4، تتراهیدروبیوپترین; AP-1, activator protein-1; ARE, antioxidant response element; BH4, tetrahydrobiopterin; Cavs, caveolins; CO, carbon monoxide; CO-RMs, CO-releasing molecules; CO3−, carbonate radical; cGMP, cyclic 3′:5′-guanosine monophosphate; DDAH, dimethylarginine dimet
A comparison of phenylketonuria with attention deficit hyperactivity disorder: Do markedly different aetiologies deliver common phenotypes?
Keywords: BH4، تتراهیدروبیوپترین; ADHD, attention deficit/hyperactivity disorders; BAEP, brainstem auditory evoked potentials; BH4, tetrahydrobiopterin; BIS, Behavioural Inhibition System; CRT, choice reaction time; DAT1, dopamine transporter 1; DLPFC, dorsolateral prefrontal cortex; DRD4
Increased expression of tryptophan-5-hydroxylase 1, but not 2, in brainstem as a result of intrauterine malnutrition
Keywords: BH4، تتراهیدروبیوپترین; IUM, intrauterine malnutrition; TPH, tryptophan-5-hydroxylase; 5-HT, 5-hydroxytryptamine; CNS, central nervous system; BH4, tetrahydrobiopterin; l-Trp, l-tryptophan; FLT, free fraction of l-Trp; BBB, blood–brain barrier; cAMP, cyclic adenosine monophospha
Arginase inhibition alleviates hypertension associated with diabetes: Effect on endothelial dependent relaxation and NO production
Keywords: BH4، تتراهیدروبیوپترین; ANOVA, analysis of variance; BH4, tetrahydrobiopterin; eNOS, endothelial nitric oxide synthase; NO, nitric oxide; PE, phenylephrine; ROS, reactive oxygen species; SNP, sodium nitroprussideDiabetes; Aorta; Hypertension; Arginase
Rapid clearance of supplemented tetrahydrobiopterin is driven by high-capacity transporters in the kidney
Keywords: BH4، تتراهیدروبیوپترین; BH4, tetrahydrobiopterin; 6RBH4, (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin dihydrochloride; BH2, 7,8-dihydrobiopterin; BP, tissue biopterin (BH2 + BH4) including a trace of fully oxidized biopterin; CsA, cyclosporin A; RBC, red blood cellTetrahydrobiopte
Novel therapeutic strategies in the management of arterial hypertension
Keywords: BH4، تتراهیدروبیوپترین; ACE, angiotensin converting enzyme; Ang, Angiotensin; ARBs, angiotensin receptor blockers; AT2R, angiotensin T2 receptor; BH4, Tetrahydrobiopterin; BP, blood pressure; ED, endothelial dysfunction; eNOS, endothelial nitric-oxide synthase; PDE5Is, phosphodi
Endothelium-dependent nitroxyl-mediated relaxation is resistant to superoxide anion scavenging and preserved in diabetic rat aorta
Keywords: BH4، تتراهیدروبیوپترین; ACh, acetylcholine; AS, Angeli's salt; 4-AP, 4-aminopyridine; BH4, tetrahydrobiopterin; cGMP, cyclic guanosine monophosphate; DEA, DEANONOate; DPI, diphenylene iodonium; EA, ethacrynic acid; EDHF, endothelium-derived hyperpolarizing factor; eNOS, endothel
Postprandial hyperglycemia on vascular endothelial function: mechanisms and consequences
Keywords: BH4، تتراهیدروبیوپترین; ADMA, asymmetric NG,NG-dimethylarginine; apoE, apolipoprotein E; AUC, area under the curve; AUCpp, postprandial area under the curve; BAEC, bovine aortic endothelial cells; BH4, tetrahydrobiopterin; CRP, C-reactive protein; CVD, cardiovascular disease; DD
HIV-1, reactive oxygen species, and vascular complications
Keywords: BH4، تتراهیدروبیوپترین; AIDS, acquired immunodeficiency syndrome; AP-1, activator protein-1; APC, antigen-presenting cells; APV, amprenavir; ARV, antiretrovirals; AZT, azidothymidine; BH4, tetrahydrobiopterin; CySS, cystine; d4T, stavudine; ddC, 2′,3′-dideoxycytidine; d-ROM, der
Age-related changes in endothelial function and blood flow regulation
Keywords: BH4، تتراهیدروبیوپترین; ACh, acetylcholine; Aβ, amyloid-β peptide; APP, amyloid precursor protein; AT2, angiotensin II type 2; BK, bradykinn; BH4, tetrahydrobiopterin; Cyclic GMP, cyclic guanosine monophosphate; ED, erectile dysfunction; EPC, endothelial progenitor cell; eNOS, e
The 48-hour tetrahydrobiopterin loading test in patients with phenylketonuria: Evaluation of protocol and influence of baseline phenylalanine concentration
Keywords: BH4، تتراهیدروبیوپترین; BH4, Tetrahydrobiopterin; BLT, BH4-loading test; Phe, Phenylalanine; PAH, Phenylalanine-hydroxylase; PKU, PhenylketonuriaPhenylketonuria; PKU; Tetrahydrobiopterin; BH4; Sapropterin; Phenylalanine
Tetrahydrobiopterin Therapy for Phenylketonuria in Infants and Young Children
Keywords: BH4، تتراهیدروبیوپترین; BH4, Tetrahydrobiopterin; OFC, Occipital-frontal circumference; PAH, Phenylalanine hydroxylase; Phe, Phenylalanine; PKU, Phenylketonuria
The emerging roles of nitric oxide (NO) in plant mitochondria
Keywords: BH4، تتراهیدروبیوپترین; AOX, alternative oxidase; BH4, tetrahydrobiopterin; DAF-2DA, 4 5-diaminofluorescein diacetate; GDC, glycine decarboxylase complex; NOS, nitric oxide synthase; l-NAME, NG-nitro-l-arginine methyl ester; l-NIL, l-N6-(1-iminoethyl)-lysine acetate; NO, nitric
Diagnosis, classification, and genetics of phenylketonuria and tetrahydrobiopterin (BH4) deficiencies
Keywords: BH4، تتراهیدروبیوپترین; BH4, tetrahydrobiopterin; CNS, central nervous system; DHPR, dihydropteridine reductase; DBS, dried blood spot; HPA, hyperphenylalaninemia; KOUT, 1st order rate of metabolic disposal; MHP, mild HPA; PAH, phenylalanine hydroxylase; PBW, percent body weight
Disorders of BH4 metabolism and the treatment of patients with 6-pyruvoyl-tetrahydropterin synthase deficiency in Taiwan
Keywords: BH4، تتراهیدروبیوپترین; Hyperphenylalaninemia; 6-pyruvoyl-tetrahydropterin synthase; Tetrahydrobiopterin; BH4; Treatment; OutcomeAR, aldose reductase; BH4, tetrahydrobiopterin; CR, carbonyl reductase; DHPR, dihydropteridine reductase; GTP, guanosine triphosphate; GTPCH, guanosin
Sex differences in the expression of serotonin-synthesizing enzymes in mouse trigeminal ganglia
Keywords: BH4، تتراهیدروبیوپترین; migraine; tryptophan hydroxylase; aromatic amino acid decarboxylase; estrus cycle; trigeminal neuron; painAADC, aromatic amino acid decarboxylase; BH4, tetrahydrobiopterin; CGRP, calcitonin gene-related peptide; DRG, dorsal root ganglion; GOI, gene of int
Immune system to brain signaling: Neuropsychopharmacological implications
Keywords: BH4، تتراهیدروبیوپترین; ACC, anterior cingulate cortex; ACTH, adrenocorticotropic hormone; BCG, Bacille Calmette–Guerin; BDNF, brain derived neurotrophic factor; BH4, tetrahydrobiopterin; BMI, body mass index; CNS, central nervous system; COX, cyclooxygenase; CRH, corticotropin-
Kearns-Sayre syndrome: Cerebral folate deficiency, MRI findings and new cerebrospinal fluid biochemical features
Keywords: BH4، تتراهیدروبیوپترین; BH4, tetrahydrobiopterin; CSF, cerebrospinal fluid; 5-HIAA, 5-hydroxyindoleacetic acid; HPLC, high performance liquid chromatography; HVA, homovanillic acid; KSS, Kearns-Sayre syndrome; 5-MTHF, 5-methyltetrahydrofolate; 5-MTHFR, 5-methyltetrahydrofolate r