Keywords: HPA; hyperphenylalaninemia; PAH; phenylalanine hydroxylase; BH4; tetrahydrobiopterin; NGS; next generation sequencing; MLPA; multiplex ligation-dependent probe amplification; qRT-PCR; quantitative real-time PCR; APV; allelic phenotype values; GPV; genotyp
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Keywords: PKU mouse model; Phenylalanine ammonia lyase; Erythrocytes as drug delivery system; Enzyme replacement therapy; Cognition and PKU; Behavior and PKU; PKU; phenylketonuria; PAH; phenylalanine hydroxylase; Phe; l-phenylalanine; Tyr; l-tyrosine; BH4; tetrahyd
Keywords: Ach; acetylcholine; ARE; antioxidant response element; BH4; Tetrahydrobiopterin; DMNQ; 2,3-Dimethoxy-1,4-naphthoquinone; EDRF; Endothelium-derived relaxing factor; eNOS; Endothelial nitric-oxide synthase; GAPDH; glyceraldehyde 3-phosphate dehydrogenase; G
Keywords: NO; nitric oxide; SNP; sodium nitroprusside; TNG; nitroglycerine; SHR; spontaneously hypertensive rats; TERPY; [Ru(terpy)(bdq)NO]3+; Eâ; endothelium-denuded; E+; endothelium-intact; 2K; sham-operated rats; 2K-1C; two-kidney one-clip, renal hypertensive
Keywords: AT-MSCs; adipose tissue-derived MSCs; BAT; brown adipose tissue; BH4; tetrahydrobiopterin; BMP; bone morphogenetic protein; DHEA; dehydroepiandrosterone; eWAT; epididymal white adipose tissue; FAs; fatty acids; G3PHD; glycerol 3-phosphate dehydrogenase; H
Keywords: Phenylketonuria; Sapropterin dihydrochloride; BH4; Intelligence; IQ; White matter; Diffusion tensor imaging;
Keywords: PKU; Phenylketonuria; phe; phenylalanine; %fatmass; percent body fat mass; %FFM; percent fat free mass; P:E ratio; protein to energy ratio; BH4; tetrahydrobiopterin; AAF; amino acid formula; E%BMR; energy intake as a percentage of basal metabolic rate; BI
Keywords: PKU; phenylketonuria; Phe; phenylalanine; PAH; phenylalanine hydroxylase; ACMG; American College of Medical Genetics and Genomics; US; United States; BH4; tetrahydrobiopterin; GMDI; Genetic Metabolic Dietitians International; Phenylketonuria; Adherence; B
Keywords: ACh; acetylcholine; ApoB100; apolipoprotein B100; B2M; β2-microglobulin; BH4; tetrahydrobiopterin; CaM; calmodulin; DHE; dihydroxyethidine; E+; ethidium; eNOS; endothelial nitric oxide synthase; EPR; electron paramagnetic resonance; FAD; flavin adenine d
Keywords: ARBs; angiotensin II type 1 receptor blockers; BH4; tetrahydrobiopterin; BMI; body mass index; CABG; coronary artery bypass graft; EPCs; endothelial progenitor cells; ERα; estrogen receptor alpha; ERβ; estrogen receptor beta; GLUT4; glucose transporter
Keywords: ADMA; asymmetric dimethylarginine; AGE; advanced glycation end-products; BH4; tetrahydrobiopterin; CAC; circulating angiogenic cells; CFU/Hill; colony forming unit-Hill; CREB; c-AMP response element-binding protein; CXCR-4; C-X-C chemokine receptor type 4
Keywords: ACEi/ARBs; angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers; ACEi; angiotensin converting enzyme inhibitors; ARB; angiotensin receptor blockers; ASE; American Society of Echocardiography; BB; beta-blockers; BH4; tetrahydrobiopteri
Keywords: SDF-1; stromal cell-derived factor 1; PAD; peripheral arterial disease; GPx; glutathione peroxidase; TAK1; transforming growth factor-B-activated-kinase 1; PTP1B; protein tyrosine phosphatase 1B; EDRF; endothelial-derived relaxing factor (EDRF); BH4; tetr
Keywords: BH4; Tetrahydrobiopterin; CSF; Cerebrospinal fluid; HVA; Homovanillic acid; L-Dopa; L-3,4-dihydroxyphenylalanine; PRL; Prolactin; UPDRS; Unified Parkinson's Disease Rating Scale;
Keywords: AGE; advanced-glycation endproducts; BH4; tetrahydrobiopterin; BKCa; large conductance calcium-activated potassium channel; COX; cyclooxygenase; EDH; endothelium-derived hyperpolarisation; eNOS; endothelial nitric oxide synthase; GHB; glycated haemoglobin
Keywords: BH4; (6R)-5,6,7,8-Tetrahydrobiopterin dihydrochloride; DPPG; 1,2-dipalmitoyl-sn-glycero-3-phospho-(1â²-rac-glycerol); DTC-Fe; dithiocarbamate-iron complex; DTT; dithiothreitol; EDTA; ethylenediaminetetraacetic acid, dipotassium salt dihydrate; EPR; elect
Keywords: ADMA; asymmetric dimethylarginine; ALP; alkaline phosphatase; ALT; alanine aminotransferase; AST; aspartate aminotransferase; BH2; dihydrobiopterin; BH4; tetrahydrobiopterin; BW; body weight; CTRL; isocaloric control standard chow diet; DEXA; dual-energy
Keywords: AGEs; advanced glycation end products; LA; alpha lipoic acid; BAEC; bovine aortic endothelial cells; BCA; bicinchoninic acid; BH4; tetrahydrobiopterin; CaI; calcium ionophore; CAT; catalase (EC 1.11.1.6); CMD; cardiometabolic disease; CVD; cardiovascular
Keywords: 5-HIAA; 5-hydroxyindolacetic acid; 5-HT; 5-hydroxytryptamine; 5-HTP; 5- hydroxytryptophan; AADC; aromatic L-amino acid decarboxylase; ADR; acute dystonic reaction; BH4; tetrahydrobiopterin; CSF; cerebrospinal fluid; DRD; dopa-responsive dystonia; dRN; dor
Keywords: Phenylketonuria; Kuvan; Sapropterin dihydrochloride; BH4; Adherence;
Keywords: BH4; 6R-5,6,7,8-tetrahydrobiopterin; BMI; body mass index; Ca; calcium; CPKU; classic PKU; Fe; iron; 25-OHD; 25-hydroxy vitamin D; MPKU; mild-moderate PKU; MHPA; mild hyper-Phe; Palb; prealbumin; Phe; phenylalanine; P; phosphorus; Se; selenium; TP; total
Keywords: BH4; tetrahydrobiopterin; BP; biopterin; i.pl.; intraplantarily; L-NAME; NÏ-Nitro-l-arginine methyl ester hydrochloride; L-NNA; NÏ-Nitro-L-arginine; NO; nitric oxide; NOS; nitric oxide synthase; PGE2; prostaglandin E2; TRPV; transient receptor potential
Keywords: BH4; tetrahydrobiopterin; DSF; differential scanning fluorimetry; L-Dopa; (S)-3,4-dihydroxyphenylalanine; PAH; phenylalanine hydroxylase; PKU; phenylketonuria; TH; tyrosine hydroxylase; THD; TH deficiency; TPH2; tryptophan hydroxylase isoform 2; PD; Parki
Keywords: FEP; first-episode psychosis; PCR; polymerase chain reaction; PANSS; Positive and Negative Syndrome Scale; GABRR2; gamma-aminobutyric acid (GABA) receptor, rho 2; GABA; gamma-aminobutyric acid; mRNA; messenger ribonucleic acid; RNA; ribonucleic acid; qPCR
Keywords: amino-BH4; 4-amino-(6R)-5,6,7,8-tetrahydro-l-biopterin; BAECs; bovine aortic endothelial cells; BH2; 7,8-dihydro-l-biopterin; BH4; (6R)-5,6,7,8-tetrahydro-l-biopterin; cGMP; 3â²,5â²-cyclic guanosine monophosphate; DAHP; 2,4-diamino-6-hydroxypyrimidine;
Keywords: AKAPs; A-kinase anchoring proteins; AKT; protein kinase b; Ang-II; angiotensin II; ASK1; apoptosis signal-regulating kinase 1; BH4; tetrahydrobiopterin; CaMKII; Ca/calmodulin-dependent kinase II; cGMP; cyclic guanosine monophosphate; CHF; chronic heart fa
Keywords: BH4; 5,6,7,8-tetrahydrobiopterin; DA; Dopamine; l-DOPA; l-3,4-dihydroxyphenylalanine; GCH; GTP cylohydrolase I; HIV; human immunodeficiency virus infection; HUVEC; human umbilical vein endothelial cells; IDO; indoleamine 2,3-dioxygenase; IFN-α -à -γ; i
Keywords: 5-HT; 5-hydroxytryptophan; 5-HTTLPR; serotonin transporter linked polymorphic region; 8-iso; 8-iso-prostaglandin F2; 8-OHdG; 8-hydroxy-2â²-deoxyguanosine; ATP; adenosine triphosphate; BH4; 5,6,7,8-tetrahydrobiopterin; CMI; cell mediated immune; CRH; cort
Keywords: 3-NT; 3-nitrotyrosine; ADMA; asymmetric dimethyl arginine; Akt; protein kinase B; AMPK; AMP-activated protein kinase; BAT; brown adipose tissue; BH2; dihydrobiopterin; BH4; tetrahydrobiopterin; BMR; basal metabolic rate; Cav-1; caveolin-1; CCK; cholecysto
Keywords: AAV; adeno-associated virus; ACMG; American College of Medical Genetics and Genomics; AHRQ; Agency for Healthcare Research and Quality; BH4; tetrahydrobiopterin; BMD; bone mineral density; CDE; Common Data Element; CHD; congenital heart disease; CNS; cent
Keywords: BH4; 6R-tetrahydrobiopterin; ECT; early and continuously treated; HPA; hyperphenylalaninemia; IDC; index of dietary control; IQ; intelligence quotient; LNAA; large neutral amino acid; PAH; phenylalanine hydroxylase; PKU; phenylketonuria; SD; standard devi
Keywords: ATGL; adipose triglyceride lipase; ATGL(â/â); adipose triglyceride lipase knockout; BH2; dihydrobiopterin, [2-amino-6-(1,2-dihydroxypropyl)-7,8-dihydro-1H-pteridin-4-one]; BH4; tetrahydrobiopterin, [(6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8
Keywords: DAH7PS; 3-deoxy-D-arabino-heptulosonate 7-phosphate synthase; IEC; ion exchange chromatography; MBP; maltose binding protein; PS; Phenyl Sepharose; Phe; phenylalanine; PAH; phenylalanine hydroxylase; PKU; phenylketonuria; PAGE; polyacrylamide gel electrop
Keywords: A; mitral inflow late filling velocity in pulse-wave Doppler; BDM; 2,3-butanedione monoxime; BH4; tetrahydrobiopterin; DOCA; deoxycorticosterone acetate; E; mitral inflow early filling velocity in pulse-wave Doppler; EF; ejection fraction; FS; fractional
Keywords: AKAP; A-kinase anchor protein; Ang II; angiotensin II; AP; action potential; APD; action potential duration; BH4; tetrahydrobiopterin; cAMP; cyclic adenosine monophosphate; CaM; calmodulin; CaMKII; Ca/calmodulin-dependent protein kinase II; CaMKIIδC; cyt
Keywords: 5-HIAA; 5 hydroxyindoleacetic acid; 5-HT; serotonin; 5-HTT; 5-HT transporter; ACTH; adrenocorticotropic hormone; AIAQ; anger irritability and assault questionnaire; AMPA; α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid; BCG; bacille Calmette-Gueri
Transcriptome of the Wistar audiogenic rat (WAR) strain following audiogenic seizures
Keywords: 5-HT; Serotonin; Acsm3; Acyl-CoA synthetase medium-chain family member 3; Acsm5; Acyl-CoA synthetase medium-chain family member 5; aGPCR; G protein-coupled adhesion receptors; ANOVA; analysis of variance; AS; audiogenic seizure; BH4; tetrahidropteridina;
Three novel variants (p.Glu178Lys, p.Val245Met, p.Ser250Phe) of the phenylalanine hydroxylase (PAH) gene impair protein expression and function in vitro
Keywords: BH4; Tetrahydrobiopterin; E. coli; Escherichia coli; ELISA; Enzyme-linked immunosorbent assay; hPAH; Human phenylalanine hydroxylase; IPTG; Isopropyl-β-d-thiogalactoside; kDa; Kilo Dalton; MBP; Maltose binding protein; PAH; Phenylalanine hydroxylase prot
Vitamin C for the treatment of sepsis: The scientific rationale
Keywords: ACTH; adrenocorticotropic hormone; BH4; tetrahydrobiopterin; CRRT; continuous renal replacement therapy; GR; glucocorticoid receptor; GULO; l-gulono-γ-lactone oxidase; G6PD; glucose-6-phosphate deficiency; HMGB1; high mobility group box 1; HOCL; hypo-chl
Genetic deletion of CD38 confers post-ischemic myocardial protection through preserved pyridine nucleotides
Keywords: Coronary circulation; Endothelium; Nitric oxide; Ischemia; Oxidant stress; 2'-P-(c)ADPR; 2â²-phospho (cyclic) adenosine diphosphate ribose; BH2; Dihydrobiopterin; BH4; Tetrahydrobiopterin; BSA; Bovine serum albumin; CF; Coronary flow; cGMP; Cyclic guan
Regulation of vascular tone homeostasis by NO and H2S: Implications in hypertension
Keywords: ADRF; adipocyte-derived relaxing factor; AE; anion exchanger; ATP; adenosine triphosphate; BH4; tetrahydrobiopterin; BKCa; big conductance Ca2+-sensitive K+ channel; CaM; calmodulin; cAMP; cyclic adenosine monophosphate; CAT; cysteine aminotransferase; CB
Salvianolic acid A inhibits calpain activation and eNOS uncoupling during focal cerebral ischemia in mice
Keywords: Salvianolic acid A; Stroke; Oxidative stress; Neurovascular protection; eNOS uncoupling; BH4; tetrahydrobiopterin; Ca2+; calcium; CaM; calmodulin; CaN; calcineurin; CCA; common carotid artery; CT; computed tomography; ECA; external carotid artery; eNOS; e
The NOS-like protein from the microalgae Ostreococcus tauri is a genuine and ultrafast NO-producing enzyme
Keywords: Arg; L-arginine; BH4; tetrahydrobiopterin, (6R)-5,6,7,8-tetrahydro-l-biopterin; CO; carbon monoxide; NO; nitric oxide; NOHA; NÏ-hydroxy-l-arginine; NOS; nitric oxide synthase; bacNOS; bacterial nitric oxide synthase; bsNOS; nitric oxide synthase from Bac
Manipulation of dopamine metabolism contributes to attenuating innate high locomotor activity in ICR mice
Keywords: Locomotor activity; Dopamine metabolism; Mice; L-DOPA; BH4;
Nitric oxide donor [Ru(terpy)(bdq)NO]3+ induces uncoupling and phosphorylation of endothelial nitric oxide synthase promoting oxidant production
Keywords: TERPY; [Ru(terpy)(bdq)NO]3+; eNOS; endothelial nitric oxide synthase; SHR; spontaneously hypertensive rats; Cav-1; caveolin-1; NO; nitric oxide; CaM; calmodulin; FAD; flavin adenine dinucleotide; FMN; flavin mononucleotide; BH4; (6R-)5,6,7,8-tetrahydro-L-
The Nrf2/GCH1/BH4 Axis Ameliorates Radiation-Induced Skin Injury by Modulating the ROS Cascade
Keywords: Ad-GCH1; GCH1-overexpression adenovirus; BH2; dihydrobiopterin; BH4; 5,6,7,8-tetrahydrobiopterin; ER; endoplasmic reticulum; GCH1; GTP cyclohydrolase I; NO; nitric oxide; NOS; nitric oxide synthase; Nrf2; NF-E2-related factor 2; ROS; reactive oxygen speci
Riociguat: Mode of Action and Clinical Development in Pulmonary Hypertension
Keywords: chronic thromboembolic pulmonary hypertension; pulmonary arterial hypertension; sGC stimulator; 6MWD; 6-minute walking distance; ADMA; asymmetric dimethylarginine; AE; adverse event; BH4; tetrahydrobiopterin; BMPR-2; bone morphogenetic protein receptor-2;
The International Working Group on Neurotransmitter related Disorders (iNTD): A worldwide research project focused on primary and secondary neurotransmitter disorders
Keywords: AADC; aromatic l-amino acid decarboxylase; AR/ADGTPCH; autosomal recessive/dominant GTP-cyclohydrolase deficiency; BH4; tetrahydrobiopterin; DAT; dopamine transporter; DÃH; dopamine β-hydroxylase; DHFR; dihydrofolate reductase deficiency; DHPR; dihydrop
Lead ions abrogate lipopolysaccharide-induced nitric monoxide toxicity by reducing the expression of STAT1 and iNOS
Keywords: BH4; tetrahydro-l-biopterin; CaM; calmodulin; FAD; flavin adenine dinucleotide; FMN; flavin mononucleotide; IFN; interferon; iNOS; inducible nitric oxide synthase; IL; interleukin; LPS; lipopolysaccharide; NADPH; nicotinamide adenine dinucleotide phosphat
Tetrahydrobiopterin Role in human umbilical vein endothelial dysfunction in maternal supraphysiological hypercholesterolemia
Keywords: MPH; maternal physiological hypercholesterolemia; MSPH; maternal supraphysiological hypercholesterolemia; GTP; guanosine triphosphate; GTPCH1; GTP cyclohydrolase 1; DHFR; dihydrofolate reductase; BH2; dihydrobiopterine; BH4; tetrahydrobiopterine; HUVECs;