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*  PND Association | Guanosine Triphosphate Cyclohydrolase I Deficiency
What is Guanosine Triphosphate Cyclohydrolase I Deficiency (GCH I, GTP Cyclohydrolase, GTPCH)?. Home , Diseases ... PDF/Printable Guide to Guanosine Triphosphate Cyclohydrolase I Deficiency(GCH I, GTP Cyclohydrolase, GTPCH) ... Guanosine Triphosphate Cyclohydrolase I Deficiency. Key Words: "GTP Cyclohydrolase". Access Listing: 600225. http://www.ncbi. ... Guanosine Triphosphate Cyclohydrolase I Deficiency (GTPCH) deficiency is a rare metabolic disorder which produces a lack of the ...
  http://www.pndassoc.org/diseases/gtpch.html
*  Tetrahydrobiopterin biosynthesis, regeneration and functions | Biochemical Journal
The pathway for the de novo biosynthesis of BH4 from GTP involves GTP cyclohydrolase I, 6-pyruvoyl-tetrahydropterin synthase ... In the liver at least, activity is inhibited by BH4, but stimulated by phenylalanine through the GTP cyclohydrolase I feedback ... With regard to the regulation of cofactor biosynthesis, the major controlling point is GTP cyclohydrolase I, the expression of ...
  http://www.biochemj.org/content/347/1/1
*  GTP cyclohydrolase II - Wikipedia
In enzymology, a GTP cyclohydrolase II (EC 3.5.4.25) is an enzyme that catalyzes the chemical reaction ... Other names in common use include guanosine triphosphate cyclohydrolase II, and GTP-8-formylhydrolase. This enzyme participates ... GTP + 3 H2O ⇌. {\displaystyle \rightleftharpoons }. formate + 2,5-diamino-6-hydroxy-4-(5-phosphoribosylamino)pyrimidine + ... Thus, the two substrates of this enzyme are GTP and H2O, whereas its 3 products are formate, 2,5-diamino-6-hydroxy-4-(5- ...
  https://en.wikipedia.org/wiki/GTP_cyclohydrolase_II
*  folE - GTP cyclohydrolase 1 - Escherichia coli (strain K12) - folE gene & protein
GTP cyclohydrolase I activity, zinc ion binding, queuosine biosynthetic process, tetrahydrobiopterin biosynthetic process ... IPR001474 GTP_CycHdrlase_I. IPR018234 GTP_CycHdrlase_I_CS. IPR020602 GTP_CycHdrlase_I_dom. ... IPR001474 GTP_CycHdrlase_I. IPR018234 GTP_CycHdrlase_I_CS. IPR020602 GTP_CycHdrlase_I_dom. ... "Allosteric characteristics of GTP cyclohydrolase I from Escherichia coli.". Schoedon G., Redweik U., Frank G., Cotton R.G.H., ...
  https://www.uniprot.org/uniprot/P0A6T5
*  folE - GTP cyclohydrolase 1 - Xanthomonas axonopodis pv. citri (strain 306) - folE gene & protein
GTP cyclohydrolase FolE2 (folE2), GTP cyclohydrolase 1 (folE). This subpathway is part of the pathway 7,8-dihydroneopterin ... IPR001474. GTP_CycHdrlase_I. IPR018234. GTP_CycHdrlase_I_CS. IPR020602. GTP_CycHdrlase_I_dom. ... IPR001474. GTP_CycHdrlase_I. IPR018234. GTP_CycHdrlase_I_CS. IPR020602. GTP_CycHdrlase_I_dom. ... GTP cyclohydrolase 1UniRule annotation. ,p>Manual validated information which has been generated by the UniProtKB automatic ...
  http://www.uniprot.org/uniprot/Q8PEP3
*  GTP cyclohydrolase I - Wikipedia
GTP cyclohydrolase I (GTPCH) (EC 3.5.4.16) is a member of the GTP cyclohydrolase family of enzymes. GTPCH is part of the folate ... as well as GTP cyclohydrolase I deficiency. Deficiency of GTP cyclohydrolase I can occur in a recessive and in a dominant form ... Patients with the recessive form have mutations in both alleles for GTP cyclohydrolase I. Patients present with developmental ... ISBN 0-471-39223-5. GTP Cyclohydrolase I at the US National Library of Medicine Medical Subject Headings (MeSH) GeneReviews/ ...
  https://en.wikipedia.org/wiki/GTP_cyclohydrolase_I
*  GTP cyclohydrolase IIa - Wikipedia
In enzymology, a GTP cyclohydrolase IIa (EC 3.5.4.29) is an enzyme that catalyzes the chemical reaction GTP + 3 H2O ⇌ {\ ... The systematic name of this enzyme class is GTP 8,9-hydrolase (phosphate-forming). Graham DE, Xu H, White RH (2002). "A member ... the two substrates of this enzyme are GTP and H2O, whereas its two products are 2-amino-5-formylamino-6-(5-phosphoribosylamino) ... of a new class of GTP cyclohydrolases produces formylaminopyrimidine nucleotide monophosphates". Biochemistry. 41 (50): 15074- ...
  https://en.wikipedia.org/wiki/GTP_cyclohydrolase_IIa
*  GTP cyclohydrolase deficiency; intrafamilial variation in clinical phenotype, including levodopa responsiveness | Journal of...
Increase of GTP cyclohydrolase 1 in mononuclear blood cells by stimulation: detection of heterozygotes of GTP cyclohydrolase 1 ... Identification of the GTP cyclohydrolase I (GTPCH 1) gene as the causative gene in most cases of levodopa responsive dystonia ... 1996) DOPA responsive dystonia in British patients: new mutations of the GTP cyclohydrolase 1 gene and evidence of genetic ... A family with a dominant form of partial GTP cyclohydrolase deficiency is described. Clinical severity varied from mild ...
  http://jnnp.bmj.com/content/66/1/86
*  folE - GTP cyclohydrolase 1 - Chlorobium tepidum (strain ATCC 49652 / DSM 12025 / NBRC 103806 / TLS) - folE gene & protein
IPR001474. GTP_CycHdrlase_I. IPR018234. GTP_CycHdrlase_I_CS. IPR020602. GTP_CycHdrlase_I_dom. ... IPR001474. GTP_CycHdrlase_I. IPR018234. GTP_CycHdrlase_I_CS. IPR020602. GTP_CycHdrlase_I_dom. ... GTP cyclohydrolase 1UniRule annotation. ,p>Manual validated information which has been generated by the UniProtKB automatic ... GTP cyclohydrolase IUniRule annotation. ,p>Manual validated information which has been generated by the UniProtKB automatic ...
  http://www.uniprot.org/uniprot/Q8KEA8
*  Overexpression of GTP cyclohydrolase 1 feedback regulatory protein is protective in a murine model of septic shock. | Sigma...
Overexpression of GTP cyclohydrolase 1 feedback regulatory protein is protective in a murine model of septic shock.. [Anna ... GTP cyclohydrolase 1 (GCH1) is the rate-limiting enzyme for BH4 biosynthesis. Under inflammatory conditions, GCH1 activity and ...
  https://www.sigmaaldrich.com/catalog/papers/25046538
*  Abstract 167: A Cell-Autonomous Role for Endothelial GTP Cyclohydrolase 1 and Tetrahydrobiopterin in Blood Pressure Regulation ...
We generated a novel mouse model with endothelial cell-specific deletion of GCH1, encoding GTP cyclohydrolase 1, an essential ... Abstract 167: A Cell-Autonomous Role for Endothelial GTP Cyclohydrolase 1 and Tetrahydrobiopterin in Blood Pressure Regulation ... Abstract 167: A Cell-Autonomous Role for Endothelial GTP Cyclohydrolase 1 and Tetrahydrobiopterin in Blood Pressure Regulation ... Abstract 167: A Cell-Autonomous Role for Endothelial GTP Cyclohydrolase 1 and Tetrahydrobiopterin in Blood Pressure Regulation ...
  http://atvb.ahajournals.org/content/34/Suppl_1/A167
*  Generation of an iPSC line from a patient with GTP cyclohydrolase 1 (GCH1) deficiency: HDMC0061i-GCH1 - Zurich Open Repository...
Generation of an iPSC line from a patient with GTP cyclohydrolase 1 (GCH1) deficiency: HDMC0061i-GCH1 ... Fibroblasts from a female patient carrying a heterozygous variation in GTP cyclohydrolase 1 (GCH1; OMIM: 600225; HGNC: 4193; c. ... Fibroblasts from a female patient carrying a heterozygous variation in GTP cyclohydrolase 1 (GCH1; OMIM: 600225; HGNC: 4193; c. ... Generation of an iPSC line from a patient with GTP cyclohydrolase 1 (GCH1) deficiency: HDMC0061i-GCH1. Stem Cell Research, 20: ...
  https://www.zora.uzh.ch/id/eprint/145474/
*  Corticotropin-releasing factor receptor antagonist attenuates LPS-induced increase of GTP cyclohydrolase I expression at murine...
Pretreatment with CP-154,526 attenuated the increase in expression levels of GTP cyclohydrolase I mRNA due to intraperitoneal ... Keywords: CORTICOTROPIN-RELEASING FACTOR; GTP CYCLOHYDROLASE I; LIPOPOLYSACCHARIDE; CP-154; LOCUS COERULEUS ... Pretreatment with CP-154,526 attenuated the increase in expression levels of GTP cyclohydrolase I mRNA due to intraperitoneal ... Corticotropin-releasing factor receptor antagonist attenuates LPS-induced increase of GTP cyclohydrolase I expression at murine ...
  http://booksandjournals.brillonline.com/content/journals/10.1163/156939105774647411
*  17beta-estradiol antagonizes the down-regulation of endothelial nitric-oxide synthase and GTP cyclohydrolase I by high glucose:...
... and the activity and gene expression of GTP cyclohydrolase I (GTPCH-I), a rate-limiting enzyme for BH4 synthesis. Both the gene ... 17beta-estradiol antagonizes the down-regulation of endothelial nitric-oxide synthase and GTP cyclohydrolase I by high glucose ... 17beta-estradiol antagonizes the down-regulation of endothelial nitric-oxide synthase and GTP cyclohydrolase I by high glucose ...
  https://www.semanticscholar.org/paper/17beta-estradiol-antagonizes-the-down-regulation-of-Miyazaki-Akita-Hayashi/f91fc38c182f476e501b237bcd3f5ab977fe3bf3
*  Endotoxemia-related acute kidney injury in transgenic mice with endothelial overexpression of GTP cyclohydrolase-1. - Radcliffe...
GTP cyclohydrolase-1 transgenic (Tg) mice demonstrated a significant increase in baseline urine NO-to-creatinine ratio and a ... in mouse microvascular endothelial cells and in vivo in transgenic mice with overexpression of endothelial GTP cyclohydrolase, ... GTP cyclohydrolase-1 transgenic (Tg) mice demonstrated a significant increase in baseline urine NO-to-creatinine ratio and a ... Endotoxemia-related acute kidney injury in transgenic mice with endothelial overexpression of GTP cyclohydrolase-1. ...
  https://www.rdm.ox.ac.uk/publications/105234
*  Expression of one isoform of GTP cyclohydrolase I coincides with the larval black markings of the swallowtail butterfly,...
Guanosine triphosphate cyclohydrolase I (GTPCHI) is essential for the synthesis of tetrahydrobiopterin (BH4) that is a cofactor ... Expression of one isoform of GTP cyclohydrolase I coincides with the larval black markings of the swallowtail butterfly, ... article{Futahashi2006ExpressionOO, title={Expression of one isoform of GTP cyclohydrolase I coincides with the larval black ...
  https://www.semanticscholar.org/paper/Expression-of-one-isoform-of-GTP-cyclohydrolase-I-Futahashi-Fujiwara/d2dadc5bd417ff921db834862ee1d31a357c3f9f
*  重组人GTP cyclohydrolase 1蛋白(ab114821)|Abcam中国
购买我们的重组人GTP cyclohydrolase 1蛋白。Ab114821为蛋白片段,在小麦胚芽中生产并经过ELISA, SDS ... Recombinant人GTP cyclohydrolase 1 protein. 参阅全部 GTP cyclohydrolase 1 蛋白酶. ... Defects in GCH1 are the cause of GTP cyclohydrolase 1 deficiency (GCH1D) [MIM:233910]; also known as atypical severe ... phenylketonuria due to GTP cyclohydrolase I deficiency;. GCH1D is one
  http://www.abcam.cn/recombinant-human-gtp-cyclohydrolase-1-protein-ab114821.html
*  Biochemical and functional characterization of Plasmodium falciparum GTP cyclohydrolase I | Malaria Journal | Full Text
GTP cyclohydrolase I (GCH1) is the first and the rate-limiting enzyme in the folate pathway. The amplification of the gch1 gene ... The potential to use the assay for inhibitor screening was validated by 8-oxo-GTP, a known GTP analogue inhibitor. ... The inhibitor test against P. falciparum GCH1 is now possible as indicated by the inhibitory effect by 8-oxo-GTP. Genetic ... activity was determined by the measurement of fluorescent converted neopterin with assay validation by using mutant and GTP ...
  https://0-malariajournal-biomedcentral-com.brum.beds.ac.uk/articles/10.1186/1475-2875-13-150
*  Regulation of β-adrenergic control of heart rate by GTP-cyclohydrolase 1 (GCH1) and tetrahydrobiopterin. - Oxford Neuroscience
Synthesis of BH4 is regulated by GTP cyclohydrolase I (GTPCH), encoded by GCH1. Recent clinical studies report associations ... Regulation of β-adrenergic control of heart rate by GTP-cyclohydrolase 1 (GCH1) and tetrahydrobiopterin. ... Synthesis of BH4 is regulated by GTP cyclohydrolase I (GTPCH), encoded by GCH1. Recent clinical studies report associations ... Regulation of β-adrenergic control of heart rate by GTP-cyclohydrolase 1 (GCH1) and tetrahydrobiopterin. ...
  https://www.neuroscience.ox.ac.uk/publications/237264
*  GTP cyclohydrolase I/BH4 pathway protects EPCs via suppressing oxidative stress and thrombospondin-1 in salt-sensitive...
We tested the hypothesis that GTP cyclohydrolase I (GTPCH I), the rate-limiting enzyme of BH4 de novo synthesis, protects EPCs ... GTP cyclohydrolase I/BH4 pathway protects EPCs via suppressing oxidative stress and thrombospondin-1 in salt-sensitive ... GTP cyclohydrolase I/BH4 pathway protects EPCs via suppressing oxidative stress and thrombospondin-1 in salt-sensitive ... Angiogenesis Inhibitors, Animals, Biopterin, Cell Adhesion, Desoxycorticosterone, GTP Cyclohydrolase, Hypertension, Male, Mice ...
  https://www.clahrc-oxford.nihr.ac.uk/publications/104793
*  GTP Cyclohydrolase/BH4 Pathway Protects EPC Function via Suppressing Oxidative Stress and Thrombospondin-1 in Salt-Sensitive...
GTP Cyclohydrolase/BH4 Pathway Protects EPC Function via Suppressing Oxidative Stress and Thrombospondin-1 in Salt-Sensitive ... GTP Cyclohydrolase/BH4 Pathway Protects EPC Function via Suppressing Oxidative Stress and Thrombospondin-1 in Salt-Sensitive ... GTP Cyclohydrolase/BH4 Pathway Protects EPC Function via Suppressing Oxidative Stress and Thrombospondin-1 in Salt-Sensitive ...
  https://www.rdm.ox.ac.uk/publications/105938
*  GCH1 gene - Genetics Home Reference
GTP-cyclohydrolase I gene mutations in patients with autosomal dominant and recessive GTP-CH1 deficiency: identification and ... GTP cyclohydrolase 1. Enable Javascript to view the expand/collapse boxes.. Printable PDF Open All Close All ... Without enough GTP cyclohydrolase 1, little or no tetrahydrobiopterin is produced. As a result, this cofactor is not available ... The GCH1 gene provides instructions for making an enzyme called GTP cyclohydrolase 1. This enzyme is involved in the first of ...
  https://ghr.nlm.nih.gov/gene/GCH1
*  Explaining the Phenomenon of Nitrate Tolerance | Circulation Research
... decrease in GTP-cyclohydrolase I feedback regulatory protein mRNA.72 This protein controls the rate of BH4 synthesis by GTP ... induced oxidation of BH4 and reduced expression of GTP-cyclohydrolase (GTP-CH); (4) Vasoconstrictor supersensitivity caused by ... cyclohydrolase (Figure 2). Provided that mRNA and protein expression are directly correlated (which we do not know), the rate ...
  http://circres.ahajournals.org/content/97/7/618