GTP cyclohydrolase
... or GTP cyclohydrolase III), part of the riboflavin and deazaflavin cofactor biosynthetic pathways GTP cyclohydrolase IV GTP ... queuosine and other biosynthetic pathways GTP cyclohydrolase Ia, GTP cyclohydrolase Ib, part of the tetrahydrobiopterin, ... Several GTP cyclohydrolases exist, which sometimes synthesize different products for different pruposes: GTP cyclohydrolase I, ... "ENZYME - 3.5.4.29 GTP cyclohydrolase IIa". enzyme.expasy.org. Retrieved 2023-01-23. (EC 3.5.4, Hydrolases). ...
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 ... autosomal recessive GTP cyclohydrolase I deficiency and autosomal dominant GTP cyclohydrolase I deficiency. These may present ... "Entrez Gene: GCH1 GTP Cyclohydrolase 1 (DOPA-Responsive Dystonia)". Šimčíková D, Heneberg P (December 2019). "Refinement of ... ISBN 0-471-39223-5. GTP+Cyclohydrolase+I at the U.S. National Library of Medicine Medical Subject Headings (MeSH) GeneReviews/ ...
GTP cyclohydrolase II
In enzymology, a GTP cyclohydrolase II (EC 3.5.4.25) is an enzyme that catalyzes the chemical reaction GTP + 3 H2O ⇌ {\ ... Other names in common use include guanosine triphosphate cyclohydrolase II, and GTP-8-formylhydrolase. This enzyme participates ... Foor F, Brown GM (1975). "Purification and properties of guanosine triphosphate cyclohydrolase II from Escherichia coli". J. ... The systematic name of this enzyme class is GTP 7,8-8,9-dihydrolase (diphosphate-forming). ...
GTP cyclohydrolase IIa
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- ...
Autosomal recessive GTP cyclohydrolase I deficiency
... (AR-GTPCHD) is a disorder associated with the deficient operation of the ... Autosomal dominant GTP cyclohydrolase I deficiency - a condition associated with mutations of the same gene, GTPCH1 Opladen T, ... As of 2020, autosomal recessive GTP cyclohydrolase I deficiency was one of the six known causes of tetrahydrobiopterin ... enzyme GTP cyclohydrolase I. The condition leads to insufficient production of the cofactor tetrahydrobiopterin necessary for ...
Autosomal dominant GTP cyclohydrolase I deficiency
... (AD-GTPCHD) is a disease caused by dysfunction of GTP cyclohydrolase I, an ... Autosomal dominant GTP cyclohydrolase I deficiency is caused by mutations in the GCH1 gene that encodes for the enzyme GTP ... Autosomal recessive GTP cyclohydrolase I deficiency Weissbach A, Pauly MG, Herzog R, Hahn L, Halmans S, Hamami F, Bolte C, ... In case of improvement, the patient would be suspected to have autosomal dominant GTP cyclohydrolase I deficiency, but would ...
GCHFR
GTP cyclohydrolase 1 feedback regulatory protein is an enzyme that in humans is encoded by the GCHFR gene. GTP cyclohydrolase I ... "Entrez Gene: GCHFR GTP cyclohydrolase I feedback regulator". Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to ... Milstien S, Jaffe H, Kowlessur D, Bonner TI (Oct 1996). "Purification and cloning of the GTP cyclohydrolase I feedback ... Nandi M, Kelly P, Vallance P, Leiper J (2008). "Over-expression of GTP-cyclohydrolase 1 feedback regulatory protein attenuates ...
Ferroptosis
January 2020). "GTP Cyclohydrolase 1/Tetrahydrobiopterin Counteract Ferroptosis through Lipid Remodeling". ACS Central Science ...
Tetrahydrobiopterin
GTP) by three chemical reactions mediated by the enzymes GTP cyclohydrolase I (GTPCH), 6-pyruvoyltetrahydropterin synthase ( ... January 2020). "GTP Cyclohydrolase 1/Tetrahydrobiopterin Counteract Ferroptosis through Lipid Remodeling". ACS Central Science ... Sapropterin is indicated in tetrahydrobiopterin deficiency caused by GTP cyclohydrolase I (GTPCH) deficiency, or 6- ... preservation of nitric oxide-mediated endothelial function in diabetes by targeted transgenic GTP-cyclohydrolase I ...
RLF (gene)
Swick L, Kapatos G (2006). "A yeast 2-hybrid analysis of human GTP cyclohydrolase I protein interactions". J. Neurochem. 97 (5 ...
Neopterin
The first being catalyzed by GTP cyclohydrolase, which opens the ribose group. Phosphatases next catalyze the hydrolysis of the ... The biosynthesis of neopterin occurs in two steps from guanosine triphosphate (GTP). ...
6-Pyruvoyltetrahydropterin synthase
Thöny B, Blau N (1997). "Mutations in the GTP cyclohydrolase I and 6-pyruvoyl-tetrahydropterin synthase genes". Human Mutation ... in the biosynthesis of tetrahydrobiopterin from GTP, which is used as a cofactor in the synthesis of aromatic amino acid ...
RAB26
"A yeast 2-hybrid analysis of human GTP cyclohydrolase I protein interactions". J. Neurochem. 97 (5): 1447-55. doi:10.1111/j. ... "cDNA cloning of a human RAB26-related gene encoding a Ras-like GTP-binding protein on chromosome 16p13.3 region". J Hum Genet. ... a low molecular weight GTP-binding protein, in the rat parotid gland". Histochem. Cell Biol. 113 (4): 259-63. doi:10.1007/ ...
PTS (gene)
Thöny B, Blau N (1997). "Mutations in the GTP cyclohydrolase I and 6-pyruvoyl-tetrahydropterin synthase genes". Hum. Mutat. 10 ...
AHSA1
Swick L, Kapatos G (2006). "A yeast 2-hybrid analysis of human GTP cyclohydrolase I protein interactions". J. Neurochem. 97 (5 ...
6-Pyruvoyltetrahydropterin synthase deficiency
Thöny B; Blau N (1997). "Mutations in the GTP cyclohydrolase I and 6-pyruvoyl-tetrahydropterin synthase genes". Hum. Mutat. 10 ...
RPH3A
Imazumi K, Sasaki T, Takahashi K, Takai Y (1995). "Identification of a rabphilin-3A-interacting protein as GTP cyclohydrolase I ...
Dopamine-responsive dystonia
Autosomal dominant GTP cyclohydrolase I deficiency (autosomal dominant Segawa syndrome) Autosomal recessive GTP cyclohydrolase ... A mutation in the gene GCH1, which encodes the enzyme GTP cyclohydrolase I, disrupts the production of BH4, decreasing dopamine ... Not all patients show mutations in the GCH1 gene (GTP cyclohydrolase I), which makes genetic testing imperfect. Sometimes a ... GeneReview/NCBI/NIH/UW entry on GTP Cyclohydrolase 1-Deficient Dopa-Responsive Dystonia GeneReview/NCBI/NIH/UW entry on ...
FolE RNA motif
All known folE RNAs are present upstream of genes encoding GTP cyclohydrolase I, which performs a step in folate metabolism. ...
Catecholamines up
In dopamine synthesis, Catsup functions to inhibit Tyrosine hydroxylase (TH), GTP Cyclohydrolase I (GTPCH), and Vesicular ... In pteridine biosynthesis pathway, Catsup negatively regulates the activity of GTP Cyclohydrolase I (GTPCH), preventing GTPCH ... GTP Cyclohydrolase I (GTPCH), and Vesicular Monoamine Transporter (VMAT) in Drosophila melanogaster. Catsup plays a significant ...
Tachykinin receptor 1
Thöny B, Heizmann CW, Mattei MG (March 1995). "Human GTP-cyclohydrolase I gene and sepiapterin reductase gene map to region ...
Sepiapterin reductase
Thöny B, Heizmann CW, Mattei MG (1995). "Human GTP-cyclohydrolase I gene and sepiapterin reductase gene map to region 14q21-q22 ...
3,4-dihydroxy-2-butanone-4-phosphate synthase
GTP cyclohydrolase II) all reside in the lux operon. RibB is sometimes found as a bifunctional enzyme with GTP cyclohydrolase ...
Tryptophan operon leader
Babitzke P, Gollnick P, Yanofsky C (1992). "The mtrAB operon of Bacillus subtilis encodes GTP cyclohydrolase I (MtrA), an ...
Lesch-Nyhan syndrome
The first step of dopamine synthesis is GTP cyclohydrolase, and significantly a deficiency of this step produces a syndrome ... Thus a lack of HGPRT may produce a nucleotide deficiency (specifically: GTP deficiency) disorder, resulting in dopamine ... However, the link between dopamine and purine synthesis is a nucleotide called guanosine triphosphate or 'GTP'. ...
2,5-Diamino-6-hydroxy-4-(5-phosphoribosylamino)pyrimidine
... formed by the hydrolysis of GTP by GTP cyclohydrolase II. Alternatively two separate enzymes can carry out this reaction, ... initially GTP cyclohydrolase IIa hydrolyses the 8,9 bond to form 2-Amino-5-formylamino-6-(5-phospho-D-ribosylamino)pyrimidin-4( ... Graham DE, Xu H, White RH (2002). "A member of a new class of GTP cyclohydrolases produces formylaminopyrimidine nucleotide ... Foor F, Brown GM (1975). "Purification and properties of guanosine triphosphate cyclohydrolase II from Escherichia coli". J. ...
Gene therapy in Parkinson's disease
This therapy consists on transferring the TH and GTP cyclohydrolase 1 genes into the MSNs because the endogenous AADC activity ... GTP cyclohydrolase 1 and AADC. A symptomatic approach is a treatment focused on the symptoms of the patients. The first one, ...
Biopterin
In the de novo pathway, GTP is converted to 7,8-dihydroneopterin triphosphate by GTP cyclohydrolase I (GTPCH-1, FolE), which ... BH4 synthesis occurs through two principal pathways; the de novo pathway involves three enzymatic steps and proceeds from GTP, ... expands the imidazole ring in GTP by one carbon. The intermediate is converted to 6-pyruvoyl-5,6,7,8-tetrahydropterin by 6- ...
GCH
... a secondary school in London GTP cyclohydrolase I, an enzyme Gym Class Heroes, an American band Knight Grand Cross of the Royal ...
International Working Group on Neurotransmitter Related Disorders
... vesicular monoamine transporter 2 GTP cyclohydrolase (Segawa disease), 6-pyruvoyl-tetrahydropterin synthase, dihydropteridine ...