Activating Compound | Comment | Organism | Structure |
---|---|---|---|
L-phenylalanine | stimulates the enzyme via mediation of GFRP which forms a complex with the enzyme | Rattus norvegicus |
Crystallization (Comment) | Organism |
---|---|
crystallization of purified recombinant enzyme and protein GFRP forming a BH2-induced inhibitory complex, involving dGTP, in 14% isopropanol, 0.2 M ammonium sulfate, 10% PEG 300, 10% ethylene glycol, 0.1 M MES-Na, pH 6.0, rapid freezing of crystals in liquid nitrogen, X-ray diffraction structure determination and analysis at 2.8 A resolution | Rattus norvegicus |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
dihydrobiopterin | induction of feedback inhibition, binding site structure | Rattus norvegicus | |
tetrahydrobiopterin | induction of feedback inhibition of the enzyme via mediation of GFRP which forms a complex with the enzyme, binding site structure | Rattus norvegicus |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Zn2+ | 1 ion per active site | Rattus norvegicus |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
50000 | - |
GFRP | Rattus norvegicus |
260000 | - |
GTP cyclohydrolase I | Rattus norvegicus |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
GTP + H2O | Rattus norvegicus | rate-limiting step in the biosynthesis of tetrahydrobiopterin, a key factor necessary for nitric oxide synthase, and for the hydrolxylases that are involved in the production of catecholamines and serotonin | formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate | - |
? | |
additional information | Rattus norvegicus | enzyme deficiency causes hyperphenylalaninemia with severe neurological disorders, the 3,4-dihydroxyphenylalanine, i.e. DOPA, responsive form of dystonia, and endothelial dysfunction, all by (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin depletion, the GTP cyclohydrolase I feedback regulatory protein GFRP binds to the enzyme and mediates the regulation of the enzyme by L-phenylalanine and (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Rattus norvegicus | - |
- |
- |
Reaction | Comment | Organism | Reaction ID |
---|---|---|---|
GTP + H2O = formate + 7,8-dihydroneopterin 3'-triphosphate | allosteric regulation mechanism, active site structure | Rattus norvegicus |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
GTP + H2O | - |
Rattus norvegicus | formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate | - |
? | |
GTP + H2O | rate-limiting step in the biosynthesis of tetrahydrobiopterin, a key factor necessary for nitric oxide synthase, and for the hydrolxylases that are involved in the production of catecholamines and serotonin | Rattus norvegicus | formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate | - |
? | |
additional information | enzyme deficiency causes hyperphenylalaninemia with severe neurological disorders, the 3,4-dihydroxyphenylalanine, i.e. DOPA, responsive form of dystonia, and endothelial dysfunction, all by (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin depletion, the GTP cyclohydrolase I feedback regulatory protein GFRP binds to the enzyme and mediates the regulation of the enzyme by L-phenylalanine and (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin | Rattus norvegicus | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
decamer | 2 pentamers which are positioned in a head-to-head manner to form the active decameric enzyme with an active site at the interface of each couple of dimer | Rattus norvegicus |
More | stimulatory and inhibitory enzyme-GFRP-complex sandwich structure, subunit composition of GFRP pentamer is betabetaalphabetabetaalphabetabeta, overview | Rattus norvegicus |
Synonyms | Comment | Organism |
---|---|---|
GTPCHI | - |
Rattus norvegicus |