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Journal Article
Journal of Human Genetics, ISSN 1434-5161, 02/2019, Volume 64, Issue 2, pp. 67 - 71
Journal Article
Annals of neurology, ISSN 0364-5134, 2012, Volume 71, Issue 4, pp. 520 - 530
Journal Article
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
Journal Article
Journal of Neuroinflammation, ISSN 1742-2094, 09/2017, Volume 14, Issue 1, pp. 181 - 15
Background: Tetrahydrobiopterin (BH4) is an essential cofactor in multiple metabolic processes and plays an essential role in maintaining the inflammatory and... 
Thrombospondin-1 | Inflammation | Tetrahydrobiopterin | BH4 | Microglia | Microvascular degeneration | VASCULAR DEVELOPMENT | MICROGLIAL ACTIVATION | ISCHEMIC RETINOPATHY | IMMUNOLOGY | NITRIC-OXIDE-SYNTHASE | NEUROSCIENCES | E-KNOCKOUT MICE | PROLIFERATIVE DIABETIC-RETINOPATHY | ENDOTHELIAL-CELLS | DISEASE | MOUSE RETINA | Inflammation - pathology | Retina - metabolism | Phenylketonurias - genetics | Retinal Vessels - metabolism | Retinal Degeneration - genetics | Mice, Inbred C57BL | Microvessels - metabolism | Microvessels - pathology | Mice, Transgenic | Retinal Degeneration - metabolism | Polycomb Repressive Complex 1 - genetics | Retinal Vessels - pathology | Inflammation - metabolism | Animals | Inflammation - genetics | Phenylketonurias - metabolism | Mice | Phenylketonurias - pathology | Retinal Degeneration - pathology | Retina - pathology | Retinal degeneration | Complications and side effects | Development and progression | Genetic aspects | Research | Diabetic retinopathy | Oxidative stress | Animal models | Retinopathy | Frizzled protein | Homeostasis | Retina | Cardiovascular disease | Interleukin 6 | Atrophy | Angiogenesis | Rodents | Microglial cells | Degeneration | Supplementation | Thrombospondin | Enzymes | Cytokines | Fetuses | Gene expression | Hyperoxia | Nitric oxide | Microvasculature | Diabetes
Journal Article
Archives of Disease in Childhood, ISSN 0003-9888, 02/2018, Volume 103, Issue 2, pp. e2 - e2
Journal Article
Circulation Research, ISSN 0009-7330, 04/2007, Volume 100, Issue 7, pp. 1089 - 1098
Journal Article
Autophagy, ISSN 1554-8627, 11/2011, Volume 7, Issue 11, pp. 1323 - 1334
Tetrahydrobiopterin (BH 4 ) deficiency is a genetic disorder associated with a variety of metabolic syndromes such as phenylketonuria (PKU). In this article,... 
Binding | Proteins | Landes | Calcium | Bioscience | Biology | Cell | Cycle | Cancer | Organogenesis | Tyrosine | Phenylketonuria | Phenylalanine | Tetrahydrobiopterin | AMPK | Akt | Autophagy | mTORC1 | tetrahydrobiopterin | autophagy | phenylalanine | MENTAL-RETARDATION | RAG GTPASES | CELL-PROLIFERATION | CELL BIOLOGY | AMINO-ACID | TYROSINE SUPPLEMENTATION | DIHYDROPTERIDINE REDUCTASE DEFICIENCY | tyrosine | MATERNAL PHENYLKETONURIA | phenylketonuria | GENETIC MOUSE MODEL | PHENYLALANINE-HYDROXYLASE DEFICIENCY | PARKINSONS-DISEASE | NIH 3T3 Cells | Liver - pathology | Biopterin - deficiency | Biopterin - pharmacology | Tyrosine - deficiency | Humans | Multiprotein Complexes | Infant | Male | Ras Homolog Enriched in Brain Protein | Biopterin - analogs & derivatives | Autophagy - drug effects | Mechanistic Target of Rapamycin Complex 1 | Liver - drug effects | Biopterin - therapeutic use | Female | Phenylketonurias - pathology | Child | Phenylalanine Hydroxylase - metabolism | Phenylalanine - metabolism | Liver - ultrastructure | Alcohol Oxidoreductases - metabolism | Neuropeptides - metabolism | Down-Regulation - drug effects | Tyrosine - metabolism | Animals | Proteins - metabolism | Monomeric GTP-Binding Proteins - metabolism | Mice | TOR Serine-Threonine Kinases | Alcohol Oxidoreductases - deficiency | Phenylketonurias - drug therapy | Basic Research Paper
Journal Article
Journal of Pediatric Endocrinology and Metabolism, ISSN 0334-018X, 08/2018, Volume 31, Issue 8, pp. 911 - 916
Journal Article