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2-amino-5-formylamino-6-ribofuranosylamino-4(3H)-pyrimidinone triphosphate + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
substrate is the reaction intermediate of the overall reaction
-
-
?
beta,gamma-methyleneguanosine 5'-triphosphate + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
beta-gamma-methyleneguanosine 5'-triphosphate + H2O
beta-gamma-methylene-7,8-dihydroneopterin 3'-triphosphate + formate
-
-
-
?
GDP + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
GDP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine diphosphate
GTP + H2O
2-amino-5-formylamino-6-ribosylamino-4(3H)-pyrimidinone triphosphate + ?
-
mutant H179N is not able to perform the whole reaction step
-
-
r
GTP + H2O
dihydroneopterin triphosphate + formate
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
GTP + H2O
formate + 7,8-dihydro-D-neopterin 2',3'-cyclic phosphate + diphosphate
-
-
-
-
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
guanosine 5'-[gamma-thio]triphosphate + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
-
-
-
?
additional information
?
-
beta,gamma-methyleneguanosine 5'-triphosphate + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
-
-
-
?
beta,gamma-methyleneguanosine 5'-triphosphate + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
-
-
-
?
GDP + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
-
-
-
?
GDP + H2O
dihydroneopterin 2',3'-cyclic phosphate + ?
-
-
-
-
?
GDP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine diphosphate
-
i.e. dihydroneopterin 3'-triphosphate
-
?
GDP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine diphosphate
-
i.e. dihydroneopterin 3'-triphosphate
-
?
GTP + H2O
dihydroneopterin triphosphate + formate
-
-
-
-
?
GTP + H2O
dihydroneopterin triphosphate + formate
-
first step in the biosynthesis of pteridine coenzymes, such as folic acid and tetrahydrobiopterin
-
-
?
GTP + H2O
dihydroneopterin triphosphate + formate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Q8S3C2
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Q8S3C2
the enzyme mediates the first and committing step pf the pterin branch of the folate-synthesis pathway
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Q8S3C2
the enzyme mediates the first and committing step of the pterin branch of the folate-synthesis pathway
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
cyclization to dihydroneopterin triphosphate
i.e. dihydroneopterin triphosphate
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
first step in the biosynthesis pathway leading to dihydrofolate and tetrahydrobiopterin
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
rate-limiting enzyme in the biosynthesis of tetrahydrobiopterin, important in the regulation of monoamine neurotransmitters such a s dopamine, norepinephrine, and serotonin
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
substrate needs to be Mg2+-free
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
the enzyme is rate limiting in the tetrahydrobiopterin biosynthesis in adipose tissue, overview
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
the enzyme synthesizes the cofactor for the reaction of the phenylalanine hydroxylase
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
first committed step in the biosynthesis of tetrahydrofolate and tetrahydrobiopterin
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
complex series of reaction steps
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
first committed step in the biosynthesis of tetrahydrofolate and tetrahydrobiopterin
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
complex series of reaction steps
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
the enzyme produces the cofactor tetrahydrobiopterin essential for activities of tyrosine hydroxylase and DOPA decarboxylase
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
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
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
estrogens play a regulatory role on the enzyme expression, the enzyme is involved in estrogen receptor interaction with cyclic AMP, role of receptor subtypes, overview
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
the enzyme controls the biosynthesis pathway of tetrahydrobiopterin, which is a necessary cofactor for inducible nitric oxide synthase
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
the enzyme is feedback regulated by the GTPCH feedback regulatory protein GFRP, overview
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Serratia indica
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Serratia indica
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Serratia indica
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
Serratia indica
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
the enzyme mediates the first and committing step of the pterin branch of the folate-synthesis pathway
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
i.e. dihydroneopterin 3'-triphosphate
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate
-
i.e. dihydroneopterin 3'-triphosphate
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in biosynthesis of tetrahydrobiopterin, BH4
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
-
-
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
Serratia indica
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
Serratia indica
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate
Serratia indica
-
first step in pathway of pterins
-
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
-
-
-
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
-
-
-
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
-
-
-
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
-
-
-
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
-
-
-
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
-
-
-
?
GTP + H2O
formate + 7,8-dihydroneopterin 3'-triphosphate
-
-
-
-
?
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
-
first commited step in the biosynthesis of tetrahydrofolate and tetrahydrobiopterin
-
-
?
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
-
enzyme catalyzed opening of the imidazole ring of GTP, kinetically competent reaction intermediate is 2-amino-5-formylamino-6-ribosylamino-4(3H)-pyrimidinone
-
-
?
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
-
-
-
-
?
GTP + H2O
formate + D-erythro-dihydroneopterin triphosphate
-
first step of the biosynthesis of (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin, i.e. BH4
-
-
?
additional information
?
-
-
GTPCH1 via tetrahydrobiopterin maintains normal blood pressure and endothelial function in vivo by preserving nitric oxide synthesis by endothelial NO synthase
-
-
?
additional information
?
-
-
GTP cyclohydrolase I stimulates tyrosine hydroxylase activity by increasing the maximal velocity of the enzyme and fails to block the feedback inhibition of tyrosine hydroxylase by dopamine
-
-
?
additional information
?
-
-
GCYH-I is not only the first enzyme of the tetrahydrofolate and tetrahydropterin pathways, but also the first enzyme of queuosine and archaeosine biosynthesis
-
-
?
additional information
?
-
-
GCYH-I is not only the first enzyme of the tetrahydrofolate and tetrahydropterin pathways, but also the first enzyme of queuosine and archaeosine biosynthesis
-
-
?
additional information
?
-
-
GTPCH I is the rate-limiting enzyme for de novo tetrahydrobiopterin synthesis
-
-
?
additional information
?
-
-
catalyzes the first step in the biosynthesis of methanopterin
-
-
?
additional information
?
-
-
no activity towards dGTP, ATP, GMP, ITP, fapy-GTP, or 7-methyl-GTP
-
-
?
additional information
?
-
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catalyzes the first step in the biosynthesis of methanopterin
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no activity towards dGTP, ATP, GMP, ITP, fapy-GTP, or 7-methyl-GTP
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GTPCH1 via tetrahydrobiopterin maintains normal blood pressure and endothelial function in vivo by preserving nitric oxide synthesis by endothelial NO synthase
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tetrahydrobiopterin production by GTPCH I occurs in close proximity to endothelial nitric oxide synthase
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isozyme GTPCHIa activity is involved in formation of the black markings on the larva during fourth ecdysis, pigmentation pathway overview
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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
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tetrahydrobiopterin, the cofactor required for hydroxylation of aromatic amino acids, regulates its own synthesis through feedback inhibition of the enzyme mediated by the regulatory subunit called GTP cyclohydrolase I feedback regulatory protein, i.e. GFRP
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enzyme is complexed with the GTP cyclohydrolase I feedback regulatory protein, i.e. GFRP, in a ratio of 1:2, complex formation is induced by phenylalanine and (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin
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enzyme is complexed with the GTP cyclohydrolase I feedback regulatory protein, i.e. GFRP, in a ratio of 1:2, complex formation is induced by phenylalanine and (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin, i.e. BH4
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GCH-1 interacts with many signal transduction proteins such as receptors (NKG2-A/B-actvating NK receptor and latrophilin-2), kinase (Death-associated protein kinase 3), adaptors (Rho-GTPase-activating protein 7 and Rho-guanine nucleotide exchange factor 3) and transcriptional factors (ATF-6 alpha and interferon regulatory factor 1)
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GCH1 interacts with GCH1 interacts with GTP cyclohydrolase I feedback regulatory protein, GFRP, and very long-chain specific acyl-CoA dehydrogenase in the liver, tubulin beta-2A chain in the liver and brain, DnaJ homolog subfamily A member 1 and fatty aldehyde dehydrogenase in the liver, heart and kidney and eukaryotic translation initiation factor 3 subunit I in all organs tested, overview. GCH1 associates with mitochondrial proteins and interacts with VLCAD, a mitochondrial protein
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GCH1 interacts with GCH1 interacts with GTP cyclohydrolase I feedback regulatory protein, GFRP, and very long-chain specific acyl-CoA dehydrogenase in the liver, tubulin beta-2A chain in the liver and brain, DnaJ homolog subfamily A member 1 and fatty aldehyde dehydrogenase in the liver, heart and kidney and eukaryotic translation initiation factor 3 subunit I in all organs tested, overview. GCH1 associates with mitochondrial proteins and interacts with VLCAD, a mitochondrial protein
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