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1. Nitric oxide: a major determinant of mast cell phenotype and function
by McCauley, S. D and Gilchrist, M and Befus, A. D
Nitric oxide: a major determinant of mast cell phenotype and function, 12/2005
Mast cells (MC) are important in the numerous physiological processes of homeostasis and disease. Most notably, MC are critical effectors in the development... 
nitric oxide - mast cell - tetrahydrobiopterin - leukotriene - degranulation
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2. Dopamine-dependent cytotoxicity of tetrahydrobiopterin: a possible mechanism for selective neurodegeneration in Parkinson's disease
by Choi, HJ and Kim, SW and Lee, SY and Hwang, O
Journal of Neurochemistry, ISSN 0022-3042, 07/2003, Volume 86, Issue 1, pp. 143 - 152
Parkinson's disease is a neurodegenerative disorder associated with selective loss of dopaminergic neurons in the substantia nigra. While the underlying cause... 
tetrahydrobiopterin
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3. Full Text Phenylalanine hydroxylase deficiency in Mexico: Genotype-phenotype correlations, BH 4 responsiveness and evidence of a founder effect
by Vela-Amieva, M and Abreu-González, M and González-del Angel, A and Ibarra-González, I and Fernández-Lainez, C and Barrientos-Ríos, R and Monroy-Santoyo, S and Guillén-López, S and Alcántara-Ortigoza, M.A
Clinical Genetics, ISSN 0009-9163, 07/2015, Volume 88, Issue 1, pp. 62 - 67
Phenylketonuria | Tetrahydrobiopterin | PKU | Hyperphenylalaninemia
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4. Full Text Sapropterin Is Safe and Effective in Patients less than 4-Years-Old with BH4 -Responsive Phenylalanine Hydroxylase Deficiency
by Shintaku, Haruo, MD, PhD and Ohura, Toshihiro, MD, PhD
Journal of Pediatrics, The, ISSN 0022-3476, 2014, Volume 165, Issue 6, pp. 1241 - 1244
Objective To assess the safety and efficacy of tetrahydrobiopterin therapy with sapropterin to treat tetrahydrobiopterin (BH4 )-responsive phenylalanine... 
Pediatrics | Tetrahydrobiopterin PAH Phenylalanine hydroxylase Phe Phenylalanine | TERM-FOLLOW-UP | TETRAHYDROBIOPTERIN | PEDIATRICS
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5. Tetrahydrobiopterin Scavenges Superoxide in Dopaminergic Neurons
by Nakamura, K and Bindokas, VP and Kowlessur, D and Elas, M and Milstien, S and Marks, JD and Halpern, HJ and Kang, UJ
Journal of Biological Chemistry, ISSN 0021-9258, 09/2001, Volume 276, Issue 37, pp. 34402 - 34407
Increased oxidative stresses are implicated in the pathogenesis of Parkinson's disease, and dopaminergic neurons may be intrinsically susceptible to oxidative... 
tetrahydrobiopterin
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6. Full Text The mechanism of BH 4-responsive hyperphenylalaninemia-As it occurs in the ENU1/2 genetic mouse model
by Sarkissian, Christineh N and Ying, Ming and Scherer, Tanja and Thöny, Beat and Martinez, Aurora
Human Mutation, ISSN 1059-7794, 10/2012, Volume 33, Issue 10, pp. 1464 - 1473
Phenylketonuria | Ubiquitination | Tetrahydrobiopterin | Misfolding
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7. Full Text Phase I clinical evaluation of CNSA-001 (sepiapterin), a novel pharmacological treatment for phenylketonuria and tetrahydrobiopterin deficiencies, in healthy volunteers
by Smith, Neil and Longo, Nicola and Levert, Keith and Hyland, Keith and Blau, Nenad
Molecular Genetics and Metabolism, ISSN 1096-7192, 04/2019, Volume 126, Issue 4, pp. 406 - 412
Tetrahydrobiopterin (BH ) is the natural cofactor of aromatic amino acid hydroxylases and essential for degradation of phenylalanine and synthesis of... 
Sepiapterin, tetrahydrobiopterin, BH4, Phenylketonuria | PKU | CNSA-001 | Primary BH4 deficiency | Phenylketonuria | Sepiapterin, tetrahydrobiopterin, BH | deficiency | Primary BH
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8. Full Text Transformation of 6-tetrahydrobiopterin in aqueous solutions under UV-irradiation
by Telegina, Taisiya A and Lyudnikova, Tamara A and Buglak, Andrey A and Vechtomova, Yulia L and Biryukov, Mikhail V and Demin, Vladimir V and Kritsky, Mikhail S
Journal of Photochemistry & Photobiology, A: Chemistry, ISSN 1010-6030, 03/2018, Volume 354, pp. 155 - 162
Melanogenesis disturbance leads to several pathologies, including vitiligo disease. Ultraviolet (UV) narrowband phototherapy (308 or 311 nm) is used in... 
Photooxidation of tetrahydrobiopterin | Vitiligo phototherapy | Autoxidation of tetrahydrobiopterin | Azacyclobutane dimer | Ultraviolet irradiation | Tetrahydrobiopterin | OXIDATIVE STRESS | AUTOXIDATION | VITILIGO | TETRAHYDROPTERIDINES | CHEMISTRY, PHYSICAL | H2O2 | BIOPTERIN | GENERATION | MOLECULAR-OXYGEN | PHOTOOXIDATION | Sapropterin dihydrochloride | Phototherapy
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9. Full Text Exploratory study of the effect of one week of orally administered CNSA-001 (sepiapterin) on CNS levels of tetrahydrobiopterin, dihydrobiopterin and monoamine neurotransmitter metabolites in healthy volunteers
by Smith, Neil and Longo, Nicola and Levert, Keith and Hyland, Keith and Blau, Nenad
Molecular Genetics and Metabolism Reports, ISSN 2214-4269, 12/2019, Volume 21, p. 100500
Tetrahydrobiopterin (BH ) is a cofactor for the enzymes tyrosine hydroxylase and tryptophan hydroxylase, the rate-limiting enzymes in the production of the... 
Phenylketonuria | Sepiapterin | Tetrahydrobiopterin | Tetrahydrobiopterin deficiencies | Biogenic amines
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10. Expression of GTP cyclohydrolase I in murine locus ceruleus is enhanced by peripheral administration of lipopolysaccharide
by Kaneko, YS and Ikemoto, K and Mori, K and Nakashima, A and Nagatsu, I and Ota, A
Brain Research, ISSN 0006-8993, 02/2001, Volume 890, Issue 2, pp. 203 - 210
Among the enzymes involved in the system for catecholamine biosynthesis, GTP cyclohydrolase I (GCH) contributes to the system as the first and rate-limiting... 
tetrahydrobiopterin
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11. Full Text 6R-tetrahydrobiopterin treated PKU patients below 4years of age: Physical outcomes, nutrition and genotype
by Aldámiz-Echevarría, Luis and Bueno, María A and Couce, María L and Lage, Sergio and Dalmau, Jaime and Vitoria, Isidro and Llarena, Marta and Andrade, Fernando and Blasco, Javier and Alcalde, Carlos and Gil, David and García, María C and González-Lamuño, Domingo and Ruiz, María A and Ruiz, Mónica and Peña-Quintana, Luis and González, David and Sánchez-Valverde, Felix
Molecular Genetics and Metabolism, ISSN 1096-7192, 05/2015, Volume 115, Issue 1, pp. 10 - 16
Phenylalanine-restricted diets have proven effective in treating phenylketonuria. However, such diets have occasionally been reported to hinder normal... 
Phenylketonuria | Tetrahydrobiopterin | Growth | Physical outcomes
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12. Full Text Anthropomorphic measurements and nutritional biomarkers after 5 years of BH 4 treatment in phenylketonuria patients
by Evers, Roeland A.F and van Wegberg, Annemiek M.J and van Dam, Esther and de Vries, Maaike C and Janssen, Mirian C.H and van Spronsen, Francjan J
Molecular Genetics and Metabolism, ISSN 1096-7192, 08/2018, Volume 124, Issue 4, pp. 238 - 242
Objective: The objective of this study was to assess the long-term effects of BH treatment on anthropomorphic measurements and nutritional biomarkers in... 
Phenylketonuria | Diet | Nutrition | Anthropomorphic measurements | Tetrahydrobiopterin
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13. Down-regulation of GTP cyclohydrolase I and tetrahydrobiopterin by melatonin
by Jang, YJ and Hong, HN and Lee, JD and Hwang, O
Neuroreport, ISSN 0959-4965, 11/2000, Volume 11, Issue 16, pp. 3627 - 3630
Tetrahydrobiopterin (BH4) is spontaneously released and extracellularly exerts a toxic effect preferentially on catecholamine cells. Its synthesis rate is... 
tetrahydrobiopterin
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14. Full Text LC determination of biopterin reduced forms by UV-photogeneration of biopterin and fluorimetric detection
by Espinosa-Mansilla, A and de la Peña, A. Muñoz and Cañada-Cañada, F and de Llanos, A. Mancha
Talanta, ISSN 0039-9140, 2008, Volume 77, Issue 2, pp. 844 - 851
An off-line photoirradiation LC fluorimetric method to determine tetrahydrobiopterin (BH ), by photogeneration of biopterin (BIO), is described, as an... 
Photoirradiation | Serum | Biopterin (BIO) | Tetrahydrobiopterin (BH 4) | Fluorimetric detection | Hyperphenylalaninemia | Tetrahydrobiopterin (BH | Tetrahydrobiopterin (BH4) | REGRESSION | CHEMISTRY, ANALYTICAL | PLASMA | LC | TETRAHYDROBIOPTERIN | Hydrochloric acid | Antioxidants | Hydrogen peroxide | Analysis
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15. Full Text Genetics of Phenylketonuria: Then and Now
by Blau, Nenad
Human Mutation, ISSN 1059-7794, 06/2016, Volume 37, Issue 6, pp. 508 - 515
ABSTRACT More than 950 phenylalanine hydroxylase (PAH) gene variants have been identified in people with phenylketonuria (PKU). These vary in their... 
tetrahydrobiopterin | phenylalanine hydroxylase | PAH | PKU | phenylketonuria | hyperphenylalaninaemia | sapropterin | Sapropterin | Phenylketonuria | Phenylalanine hydroxylase | Tetrahydrobiopterin | Hyperphenylalaninaemia | IN-VITRO EXPRESSION | GENOTYPE-PHENOTYPE CORRELATIONS | MILD PHENYLKETONURIA | PHARMACOLOGICAL CHAPERONES | STRUCTURAL-CHARACTERIZATION | DIHYDROPTERIDINE REDUCTASE DEFICIENCY | GENETICS & HEREDITY | LOCUS-SPECIFIC KNOWLEDGEBASE | TETRAHYDROBIOPTERIN DEFICIENCY | INTERALLELIC COMPLEMENTATION | PHENYLALANINE-HYDROXYLASE DEFICIENCY | Phenylalanine Hydroxylase - genetics | Phenotype | History, 21st Century | Phenylketonurias - genetics | Phenylketonurias - history | History, 20th Century | Humans | Phenylketonurias - metabolism | Mutation | Biopterin - analogs & derivatives | Phenylalanine Hydroxylase - metabolism | Biopterin - metabolism | Genetic aspects | Hydroxylases | Phenylalanine | Gene mutations | Genotype & phenotype | Amino acids | Genetic disorders
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16. Full Text Stoichiometric relationships between endothelial tetrahydrobiopterin, endothelial NO synthese (eNOS) activity, and eNOS coupling in vivo: Insights from transgenic mice with endothelial-targeted GTP cyclohydrolase 1 and eNOS overexpression
by Bendall, Jennifer K and Alp, Nicholas J and Warrick, Nicholas and Cai, Shijie and Adlam, David and Rockett, Kirk and Yokoyama, Mitsuhiro and Kawashima, Seinosuke and Channon, Keith M
Circulation Research, ISSN 0009-7330, 10/2005, Volume 97, Issue 9, pp. 864 - 871
Endothelial nitric oxide synthase | Superoxide | Tetrahydrobiopterin | Nitric oxide
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17. Full Text Anthropomorphic measurements and nutritional biomarkers after 5 years of BH4 treatment in phenylketonuria patients
by Evers, Roeland A.F and van Wegberg, Annemiek M.J and van Dam, Esther and de Vries, Maaike C and Janssen, Mirian C.H and van Spronsen, Francjan J
Molecular Genetics and Metabolism, ISSN 1096-7192, 08/2018, Volume 124, Issue 4, pp. 238 - 242
The objective of this study was to assess the long-term effects of BH treatment on anthropomorphic measurements and nutritional biomarkers in phenylketonuria... 
Phenylketonuria | Diet | Nutrition | Tetrahydrobiopterin | Anthropomorphic measurements | MEDICINE, RESEARCH & EXPERIMENTAL | THERAPY | ENDOCRINOLOGY & METABOLISM | GENETICS & HEREDITY | TETRAHYDROBIOPTERIN BH4 | CHILDREN | AGE
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18. Full Text Pterins as diagnostic markers of exercise-induced stress: a systematic review
by Lindsay, Angus and Gieseg, Steven P
Journal of science and medicine in sport, ISSN 1440-2440, 08/2019
To evaluate pterins as diagnostic biomarkers of exercise-induced stress. Systematic review of the literature. MEDLINE, Scopus and Web of Science were searched... 
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19. Full Text International best practice for the evaluation of responsiveness to sapropterin dihydrochloride in patients with phenylketonuria
by Muntau, Ania C and Adams, Darius J and Bélanger-Quintana, Amaya and Bushueva, Tatiana V and Cerone, Roberto and Chien, Yin-Hsiu and Chiesa, Ana and Coşkun, Turgay and de las Heras, Javier and Feillet, François and Katz, Rachel and Lagler, Florian and Piazzon, Flavia and Rohr, Fran and van Spronsen, Francjan J and Vargas, Paula and Wilcox, Gisela and Bhattacharya, Kaustuv
Molecular Genetics and Metabolism, ISSN 1096-7192, 05/2019, Volume 127, Issue 1, pp. 1 - 11
Phenylketonuria (PKU) is an inherited metabolic disease caused by phenylalanine hydroxylase (PAH) deficiency. As the resulting high blood phenylalanine (Phe)... 
Response | Pregnancy | Phenylketonuria | Phenylalanine | Tetrahydrobiopterin | Sapropterin dihydrochloride | MEDICINE, RESEARCH & EXPERIMENTAL | ADULT PATIENTS | DIAGNOSIS | BASE-LINE | BH4 | HYPERPHENYLALANINEMIA | TETRAHYDROBIOPTERIN LOADING TEST | ENDOCRINOLOGY & METABOLISM | GENETICS & HEREDITY | MATERNAL PHENYLKETONURIA | PKU | LONG-TERM TREATMENT | PHENYLALANINE-HYDROXYLASE DEFICIENCY
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20. Full Text Inflammatory monocytes determine endothelial nitric-oxide synthase uncoupling and nitro-oxidative stress induced by Angiotensin II
by Kossmann, Sabine and Hu, Hanhan and Steven, Sebastian and Schönfelder, Tanja and Fraccarollo, Daniela and Mikhed, Yuliya and Brähler, Melanie and Knorr, Maike and Brandt, Moritz and Karbach, Susanne H and Becker, Christian and Oelze, Matthias and Bauersachs, Johann and Widder, Julian and Münzel, Thomas and Daiber, Andreas and Wenzel, Philip
Journal of Biological Chemistry, ISSN 0021-9258, 10/2014, Volume 289, Issue 40, pp. 27540 - 27550
Background: Inflammatory monocytes are drivers of vascular injury and disease. Results: Depletion of lysozyme M-positive monocytes prevents eNOS uncoupling and... 
Tetrahydrobiopterin (BH4) | Monocyte | BIOCHEMISTRY & MOLECULAR BIOLOGY | Nitric-oxide Synthase | Inflammation | CELL-LINEAGE-ABLATION | VASCULAR SUPEROXIDE-PRODUCTION | DIABETES-MELLITUS | NAD(P)H OXIDASE | INTERFERON-GAMMA | Endothelial Dysfunction | GTP-CYCLOHYDROLASE-I | GENE-EXPRESSION | TETRAHYDROBIOPTERIN | DYSFUNCTION | Angiotensin II | HUMAN ATHEROSCLEROSIS | Nitric Oxide - immunology | Nitric Oxide Synthase Type III - immunology | Angiotensin II - genetics | Oxidative Stress | Mice, Inbred C57BL | Male | Biopterin - immunology | Monocytes - immunology | Endothelium, Vascular - enzymology | Biopterin - analogs & derivatives | Nitric Oxide Synthase Type III - genetics | Mice, Knockout | Angiotensin II - immunology | Monocytes - enzymology | Animals | Nitric Oxide Synthase Type II - genetics | Nitric Oxide Synthase Type II - immunology | Mice | Endothelium, Vascular - immunology | Molecular Bases of Disease
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